![]() | Only 14 pages are availabe for public view |
Abstract Thalassemia is one of the most common single gene disorders and it is widely distributed in the Meditrerranean region . Beta thalassemia major is the term applied to patients who have either no effective production (as in homozygous β0 thalassemia) or severely limited production of beta globin. These are the patients originally described by Cooley (Cooley’s anemia). Starting dur ing the first year of life, they have profound and life-long transfusion-dependent anemia. This inherited disorder makes many complications as iron overload, cardiac, hepatic, endocrine, renal, pulmonary, vascular, skeletal changes, extramedullary hematopoiesis and blood transfusion complications. The mainstays of therapy for beta thalassemia major are chronic hypertransfusion combined with iron chelation, this leading to face complications of transfusion as hepatitis B or C transmission and liver affection. With the increasing number of patients suffering from chronic liver disease from HBV and HCV, which may progress to cirrhosis with all its complications; clinicians and patients require accurate information about the degree of liver fibrosis, to guide management decisions, monitor disease activity, and predict outcome. Liver biopsy, which is considered the ultimate standard, has three major limitations: a risk of adverse events, sampling error, and intra and inter observer variability. The factors associated with this inaccuracy of liver biopsy include: the heterogeneous nature of chronic liver disease; the relatively small size of liver biopsy sample compared to the size of liver (1/50,000), or even the size of a nodule as in macronodular cirrhosis, 120 Summary which is > 3mm; and the experience of the histopathologist. These limitations of biopsy have led clinical investigators to study alternative methods to investigate liver disease. Noninvasive markers are the most widely used alternative to liver biopsy to stage chronic liver disease. Serum markers of liver fibrosis offer an attractive alternative. They are less invasive than biopsy, with no risk of complications, eliminate sampling and observer variability. An alternative method to stage chronic liver disease is elastography, or measurement of hepatic elasticity or stiffness. Our study done on 30 thalassemic children who had HCV or HBV in which fibroscan is done and other non invasive parameters which are APRI test and Forns index to evaluate liver fibrosis or cirrhosis in these patients. The results of our study revealed that: - Our study revealed that there is highly strong correlation (p< 0.0001) between fibroscan and age ,weight, ALT, Serum albumin and prothrombin concentration while significant correlation (p < 0.05) between fibroscan and height, HC, AST and serum ferritin . - Our study also showed highly significant correlation (p <0.001) between fibroscan and (age , weight) and only significant correlation ( p< 0.05) with ( height , HC). - Our study revealed that the mean value of fibroscan showed a highly significant correlation according to liver stiffness with highest level in patients with cirrhosis . - Our results indicated that liver stiffness measurements could be used to evaluate liver fibrosis in chronic liver diseases, whatever the etiology , also it correlates with fibrosis stages with increasing reliability in cases with more extensive fibrosis or cirrhosis. Thus , fibroscan could 121 Summary be a promising non invasive method for assessing advanced fibrosis in patients with chronic liver diseases. - Our study showed that highly significant correlation (p<0.001) between APRI test and (platelet count, ALT, AST, serum ferrit in ) and significant correlation (p<0.05) between APRI test and (serum albumin & prothrombin concentration). - In our study we found that there is highly significant correlation between Forns index and platelet count while no significant correlation with other parameters . No significant correlation between Forns index and liver fibrosis. |