RESUMO
Background: Transfusion dependent thalassemia patients are reported to have Vitamin D insufficiency/deficiency in many countries. Vitamin D hydroxylation occurs in the liver; whether liver iron overload interferes with this step has not been addressed till date. This study helps to establish an association between liver iron concentration (LIC) and heart iron concentration (MIC) with vitamin D levels and Bone Mass Density in these patients.Methods: A cross sectional study was done by including transfusion dependent Thalassemia patients (TM) if they had an assessment of Liver and cardiac iron done by T2*MRI and bone mineral density by DEXA. Clinical data regarding age, gender, type of iron chelation therapy and laboratory data of S. ferritin and Vitamin D was collected. Data was assessed using appropriate statistical methods.Results: Among 40 TM patients were taken and mean age was 17.6 years. Vitamin D deficiency was identified in 26(65%). 20 out of them had an LIC>7mg/g DW and 6 had MIC>1.65mg/g DW. There was a significant association between LIC>7mg/g and vitamin D level<20 ng/ml and a significant inverse correlation between LIC and vitamin D, suggesting that liver iron overload may indeed affect vitamin D metabolism. Osteopenia was present in 32.5% and osteoporosis was present in 27.5 % of all TM patients. Reduced Bone Mass Density was also found to be linked with iron over load.Conclusions: Regular monitoring of vitamin D levels and supplementation is required in patients with severe liver and heart iron load. More studies are needed to confirm these results.
RESUMO
The patient presented with progressively increasing ascites and pain abdomen but without any bleeding tendency, or encephalopathy. The initial diagnosis of acute hepatitis was proved wrong by contrast enhanced CT scan of abdomen which showed atretic hepatic veins. Atretic hepatic veins causing Budd Chiari Syndroem (BCS) is a rarity in literature.