Abstract

17 cases of Indian childhood cirrhosis were studied for blood and urinary α-ketoacids. All the cases, except one, were in an advanced stage with evidence of hepatocellular failure. In the case without hepatocellular failure there was a considerable increase in urinary excretion of α-ketoglutaric acid while that of pyruvic acid was only slightly affected and the blood ketoacid levels remained within normal limits. The possibility of this being due to early renal tubular involvement is considered. In advanced stages the mean fasting blood α-ketoglutaric acid value fell below normal (in no case was the upper limit for normal exceeded) while that of pyruvic acid rose appreciably. This is more in line with the hypothesis of a metablic block in the Kreb's citric acid cycle. Also in the urine there was considerable rise in pyruvic acid excretion while α-ketoglutaric acid excretion, inspite of possible renal tubular involvement, was normal, which further supports the above hypothesis. In some of the patients in hepatic coma there appeared three more ketoacids in the urine in small amounts. The nature of these ketoacids is still to be determined.

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