Amino acids labelled with 11C as indicator of the effect of dietary treatment of hyperammonaemia.
Short-lived radioactive carbon, 11C, (T 1/2 = 20 min) was incorporated into an essential amino acid [11C-methyl] -L-methionine, to form a true biological amino acid tracer with external detectability. This was tested in a study of the physiological tracer dynamics in a hyperammonaemic patient before and after a change in the dietary treatment. The protein intake was unchanged between the two investigations but the energy intake was increased from 53 to 63 kcal/kg BW/day. The tracer radioactivity was given per os. In the second investigation a relative decrease of radioactivity in the low molecular weight fraction of blood plasma was seen. Also the external measurements indicated a higher hepatic retention of radioactivity in the second investigation but no increased excretion of tracer. This may reflect an increased ability of the liver to utilize the incoming methionine from the vena porta. The hyperammonaemia remained over the second investigation but seven months later the ammonia content in the blood was almost normalized and the patient had also gained 3 kg in weight. The correlation between changes in tracer dynamics and changes in therapeutical effect of the diet is not further verified in this experiment but the investigation indicates the value of further studies in this topic using 11C-labelled amino acids also including the use of the newly introduced positron tomographic technique. It may be possible to develop this type of nuclide technique further to achieve a clinically useful method of optimizing therapeutic regiments in this type of metabolic disease.
D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism*; D000596:Amino Acids / Q000378:metabolism*; D000641:Ammonia / Q000378:metabolism*; D002250:Carbon Radioisotopes; D002675:Child, Preschool; D005260:Female; D005753:Gastric Mucosa / Q000378:metabolism; D006801:Humans; D007700:Kinetics; D008099:Liver / Q000378:metabolism; D009132:Muscles / Q000378:metabolism; D011865:Radioisotope Dilution Technique; D014018:Tissue Distribution
D000596:Amino Acids; D002250:Carbon Radioisotopes; D000641:Ammonia