Nutritional factors and thalassaemia major

Abnormal growth is a common feature of thalassaemia major in children. In an attempt to determine whether it has a nutritional cause, 12 children aged 1 to 3 years with thalassaemia major were studied under metabolic ward conditions. Nutritional status was assessed by anthropometry and biochemistry before and after an intensive nutrition regimen. Five children had wasting or stunting on admission. As a result of the nutrition intervention, mean weight for height improved significantly. The mean height increase of 0.4 cm after one month was not significant. Plasma zinc, depressed in half the children on admission, improved, as did alpha tocopherol, while copper decreased. Plasma insulin-like growth factor-I also increased commensurate with improved growth. Fat absorption was normal in all children. Undernutrition is an important cause of associated growth disturbances in children with thalassaemia major. Malnutrition was primarily caused by inadequate nutrient intake, as indicated by the capacity to gain weight appropriately when provided with nutrition support, and by the absence of intestinal malabsorption. While long term studies are required to determine if nutritional support will prevent stunting, these results underscore its central role in preventing nutritional deficiencies and in promoting normal growth in thalassaemic children.     

Direct demonstration of transsynaptic degeneration in the human visual system: a comparison of retrograde and anterograde changes

Transneuronal degeneration of retinal ganglion cells was directly demonstrated in a patient who had unilateral removal of the striate cortex forty years prior to necropsy. For comparison, another case is presented showing anterograde transneuronal atrophy forty years after enucleation of one eye.     

Coagulation factors in the premature infant born after about 32 weeks of gestation

In plasma samples from 10 premature infants born after about 32 weeks of gestation, a number of coagulation factors have been determined. For 9 infants, who were healthy, mean values are given: fibrinogen-antigen, 311 mg/dl; factor II, +/- 0.46 U/ml; factor V, 0.80 U/ml; factor VII, 0.59 U/ml; factor VIII coagulant activity, 0.93 U/ml; factor VIII-related antigen, 1.66 U/ml; procoagulant factor VIII antigen, 1.15 U/ml; factor IX coagulant activity, 0.41 U/ml; factor IX antigen, 0.42 U/ml; factor X coagulant activity, 0.52 U/ml; factor X antigen, 0.61 U/ml, and antithrombin III-antigen (AT-III), 0.43 U/ml. In 5 infants a second sample was taken a week after the first one; for most values there was no significant rise, except for factor II and AT-III. The values we found in this group of premature infants are within the range of those reported in earlier literature. They are higher than the ones we found in early fetal samples and most of them are similar to those we found in early fetal samples and most of them are similar to those we found in the cord blood of full-term newborns.     

Successful pregnancy in transfusion-dependent thalassaemia

Successful pregnancy in a transfusion-dependent thalassaemic patient receiving subcutaneous desferrixaomine is reported. This is the first such case to be described.     

Inhibition of platelet function by contrast media: iopamidol and ioxaglate versus iothalamate. Work in progress

The effects of an ionic contrast agent, meglumine iothalamate (Conray-60), and two newer low-osmolality radiographic contrast media, sodium meglumine ioxaglate (Hexabrix) and iopamidol (B-15,000), on platelet aggregation and secretion responses were studied. All three agents inhibited platelet responses during stimulation with adenosine diphosphate (ADP), epinephrine, and collagen. Platelet function was inhibited by iothalamate at concentrations of 11 mg iodine/ml and above, and by the newer agents at concentrations above 30 mg iodine/ml. Addition of exogenous calcium decreased the iothalamate-induced inhibition of aggregation but did not improve dense granule secretion. There was no consistent effect of exogenous calcium on platelet inhibition by iopamidol and ioxaglate. These studies indicate that the newer agents inhibit platelet function less than iothalamate does, and that chelation of Ca2+ may not be the major mechanism of platelet inhibition by contrast agents.     

Left ventricular volumes measured by MR imaging

We assessed the potential of proton magnetic resonance (MR) imaging for accurately measuring left ventricular volumes using 15 latex casts of excised human left ventricles. The casts were submerged in water to stimulate the endocardial left ventricular cavity interface in in vivo imaging conditions. Tomographic image sections perpendicular to the long axis of the cast were obtained, spanning each cast from apex to base. Simpson's rule was used to calculate the cast volumes. Correlation between the actual cast volumes (as measured by the displacement method) and the calculated volumes using MR imaging for the 15 casts was excellent. Our data demonstrate that MR imaging accurately measures cardiac chamber volumes in this in vitro model.