Thalassaemia is one of the most common genetic disorders in India. Its control is possible by screening of general population for carrier status and by antenatal diagnosis in couples at risk of having a child with thalassaemia. This study explored the feasibility of screening the extended family to detect carriers to prevent birth of thalassaemic children and identified the barriers to its acce...

In order to characterize the defect in erythroid homeostasis in chronic inflammatory states, the relationship between erythropoietin production and erythropoietic response was examined in rats with adjuvant disease. Exposure of adjuvant-injected rats to graded levels of lowered barometric pressure induced increases in plasma erythropoietin which were significantly less than those measured in no...

We describe the methodology used for quantifying and characterizing porphyrins in various tissues and in excreta, in the diagnosis and monitoring of the therapeutic modulation of biochemical disease activity in a 53-year-old white man who has a rare form of familial porphyria cutanea tarda with bone marrow rather than hepatic expression of the disease. Liquid-chromatographic and thin-layer chro...

Cellulose acetate membranes (CAM) placed in the peritoneal cavity of mice develop a macrophage layer capable of supporting in vivo hematopoietic colonies from intraperitoneally injected bone marrow cells. Modifications allowing for routine morphologic identification of colonies showed that both erythrocytic (E) and granulocytic (G) colonies occur with a consistent E:G ratio of 0.19 +/- 0.037. S...

The role of the kidney in foetal erythropoiesis was studied in newborn SD mice on the day of birth. Some of the homozygotes and heterozygotes of this strain are born anephric. Red cell production was evaluated by haematocrit levels, reticulocyte counts, and Fe-uptake in liver and RBC, the isotope given to the mothers during pregnancy. Erythropoiesis of the newborn with renal agenesis was not di...

Regular blood transfusion followed by iron chelation therapy is just a supportive treatment for thalassemia major which is associated with serious complications. Growth disturbances are a major clinical feature of untreated patients with thalassemia. The increasing mean survival age is indicative of the fact that modern therapies are generally safe and effective but it is becoming increasingly ...

Disturbances of growth and development in patients with thalassaemia receiving hypertransfusion programmes are well recognised and are most likely to be due to iron overload. The extent of endocrine dysfunction was investigated in a group of 18 patients thought to have been treated by acceptable modern standards, 11 of whom could be considered as well chelated. Assessment of growth and puberty ...

The sedimentation velocity profiles of the entities in mouse bone marrow responsible for erythropoietic burst formation (BFU-E) and for erythrocytic colony formation (CFU-E) have been studied under conditions designed to determine whether the values observed are real or result from cell interactions occurring during culture of the fractions. Bone marrow cells of adult C3Hf/Bi mice were subjecte...