ABSTRACT
In three patients with chronic conjugated hyperbilirubinemia who carried the diagnosis of Rotor syndrome, 99mTc-HIDA cholescintigraphy was performed. In these patients, the liver was either not visualized or it was seen very faintly with slow liver uptake, persistent visualization of the cardiac blood pool and prominent kidney excretion. The present observation emphasizes the contribution of cholescintigraphy in the diagnosis of Rotor syndrome.
Subject(s)
Biliary Tract/diagnostic imaging , Hyperbilirubinemia, Hereditary/diagnostic imaging , Adolescent , Child , Child, Preschool , Humans , Imino Acids , Liver/diagnostic imaging , Male , Organotechnetium Compounds , Radionuclide Imaging , Technetium Tc 99m LidofeninABSTRACT
The effect of blood transfusion on 51Cr-red blood cells surface-counting patterns was studied in 22 patients with homozygous beta-thalassaemia being transfused during erythrocyte survival study. Surface-counting patterns of 15 thalassaemics who had not been submitted to blood transfusion during the erythrocyte survival study were served as controls. In 17 of the transfused patients (group A), spleen excess counts (SEC) appeared decreasing after blood transfusion, reaching a minimum (about 50% of the initial value) 3-4 days later, and increasing thereafter to reach again their initial value on 8th to 10th posttransfusion day. The SEC pattern of the remaining 5 transfused patients (group B) presented a continuously ascending curve. Corresponding changes were observed in excess liver counts and heart counts patterns. As a result of these changes spleen/liver ratio (S/L) was found significantly affected by blood transfusion in the majority of thalassaemic patients which were transfused during the erythrocyte survival study. Furthermore, the mean age of group A patients (12.1 +/- 5.5 years) has been found to be significantly lower than that of group B (21.8 +/- 3.3 years). On the contrary, in the control group the SEC pattern appeared permanently increasing and the S/L was found unaltered during the whole duration of the erythrocyte survival study. In conclusion, as far as the surface-counting data represent indexes for splenectomy in thalassaemic patients, the results of this study signify that the influence of blood transfusion on these has to be taken into account.
Subject(s)
Thalassemia/therapy , Blood Transfusion , Chromium Radioisotopes , Erythrocyte Aging , Female , Homozygote , Humans , Male , Splenectomy , Thalassemia/blood , Thalassemia/geneticsABSTRACT
The plasma clearance rate of heat denatured human serum albumin (DHAI-125, 5 mg/kg body weight) was studied in 20 children with homozygous-beta-thalassemia before and 7--10 days after blood transfusion. A significant increase of the DHAI-125 clearance rate (P less than 0.02) was found 7--10 days after blood transfusion while the spleen presented its minimum size. This finding may be relevant to the improved intrasplenic blood circulation after blood transfusion due to the release of the blood trapped within the spleen.
