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The causes of Budd-Chiari syndrome are quickly reviewed with reference to the pathology described in series from various parts of the world. In the West the syndrome is caused by obstruction of the Inferior Vena Cava in its retrohepatic course with or without concomitant obstruction of the suprahepatic veins attributable to fibrous regression of the vessel wall and subsequent endoluminal thrombosis. In the Far East and South Africa the syndrome is caused by the presence of a thin fibrous septum obstructing the vein immediately upstream of the right atrium. Of the two cases of Budd-Chiari syndrome described, one presented high grade stenosis of the Vena Cava caused by fibrous regression of the vessel wall and the other a fibrous septum situated transversally to the lumen. The description of the first case is particularly interesting because before the clinical picture was correctly understood, a succession of major complications induced incorrect diagnosis and treatment. In contrast the second was a classic case of rapid diagnosis and successful treatment. The discussion of the two cases highlights the primary role currently played by abdominal echography with Doppler ultrasound, examination with lower cavography using a contrast medium in documenting a caval flow pathology of this kind. CAT scanning, arteriography of the Celiac tripod and closed hepatic needle biopsy appear to be much less decisive.

The behaviour of glycaemia, insulinaemia, phosphoraemia, somatotropinaemia,free glycerol and triglyceridaemia was studied in six patients with A.L.S. following sugar load (1 g/Kg) in fasting. The results of glycaemia and insulinaemia were in tune with published data which have pointed to reduced sugar tolerance and reduced insulin secretion in patients with A.L.S. In the present experiments, particularly significant were the phosphoraemia responses. The failure of inorganic phosphorus values to fall after glucose loading suggests that the glycidic intolerance of these patients is related above all to a reduction in functioning muscular mass rather than to insufficient insulin secretion. The reduction in nervous tissue may also be of importance in this sense. In fact, not all biohumoral parameters investigated were similar to those of diabetes because the behaviour of somatotropinaemia, free glycerol and plasma triglycerides was normal. The changed behaviour of phosphorus would thus indicate altered glucose uptake at peripheral tissue level.

Red cell and haemoglobin values in 44 women with a typical picutre of anorexia nervosa showed that 20.5% presented a picture of true anaemia. Blood iron was low, sometimes very low, with a mean value of 66 mg 0/0. Clotting parameters: PTT, TT, PT, circulating platelets and TEG were normal. This finding serves to explain the low incidence of haemorrhage and the ready haemostasis noted in this disease, in spite of the considerable food deficit.

The effect of three isolated defects in the enterohepatic circulation of bile acids on the size and distribution of the bile acid pool, plasma bile acid levels and bile acid secretion into the intestine was simulated using a linear multicompartmental physiological pharmacokinetic model previously used to simulate these aspects of bile acid metabolism in healthy man. Stepwise increases in portal-systemic shunting (with a reciprocal decrease in hepatic blood flow) caused an exponential increase in systemic plasma concentrations of bile acids, but no other major changes in bile acid metabolism. When the effect of varying fractional hepatic extraction was simulated, it was found that the greater the fractional hepatic extraction, the greater the elevation observed for systemic plasma bile acid levels for a given degree of portal-systemic shunting. When total hepatic blood flow was restored to normal by simulating "arterialization," systemic plasma levels of bile acids decreased strikingly, yet remained elevated. For cholate with a fractional hepatic extraction of 0.9 and 100% portal-systemic shunting, arterialization caused a decrease from a 20-fold elevation to a 5-fold elevation. This simulation thus defined the effect of the presence of the portal venous system per se on plasma bile acid levels and also quantified the circulatory route by which substances reach the liver when portal-systemic shunting is present. An isolated defect in hepatic uptake of bile acids caused little change in overall bile acid metabolism other than modestly increased plasma levels. Loss of bile acid storage by the gallbladder caused the majority of the bile acid pool to move from the gallbladder compartments to the proximal small intestine during fasting but had little effect on the dynamics of the enterohepatic circulation during eating. The results of these novel simulations of isolated defects in bile acid transport should aid in the interpretation of the more complex changes in bile acid metabolism which are likely to occur in hepatic or biliary disease.

Data regarding the commonest biohumoral tests of liver function obtained in 53 highly obese women of more or less young age, non-diabetic, non alcolist and without cardiovascular complications are reported. Liver biopsy was carried out in 23 of these patients for the purpose of studying the liver histologically. On the whole, liver function tests gave normal results: specifically, B.S.F. clearance was normal in 91% of the cases and retention at 45' was normal in 77%. Histologically, only 9% showed normal liver parenchyma. The vast majority showed a varying extent of fat infiltration and in 30% this was also fibrosis. No relationship was found to exist between the extent of histological changes and B.S.F clearance and retention values.

The Authors illustrate the main functional parameters of the whole blood in a heterozygous carrier of the unstable Hb-Atlanta. The most important results are represented by a right-shifted P50, by a lowered value of n and by an almost normal pH-depending Bohr effect. It is very likely that the first report depends on the relative Hb-A deficiency, whereas the second one could be due to the presence of the unstable Hb. Moreover, the linkage between this latter and DPG would be present.