Metabolism studies in a case of congenital hemolytic jaundice with splenomegaly

In a case of congenital hemolytic jaundice with splenomegaly, we have found in a metabolism experiment of five days, on the Folin diet, a loss of 4.06 grammes of nitrogen, while the urinary nitrogen partition was normal in character, with the exception of the uric acid nitrogen, which was increased. The absorption of nitrogen was normal.

The urinary sulphur partition was normal in character with occasional increased excretions of ethereal sulphates. In the five days, there was a loss of 18.8 grammes of sulphur, 0.482 grammes of calcium oxide and 0.924 grammes of magnesium oxide. There was a phosphorus retention of 0.07 grammes, while the amounts of earthy phosphates and total phosphates may be considered normal.

There was a loss of 0.1199 grammes of iron, with marked increased amounts of iron excreted in the urine and feces. The fat metabolism was normal, with an absorption of about 91 per cent. of the ingested fat. The amounts of neutral fat, fatty acids and soaps in the stool were normal.

A marked disturbance in the cholesterol metabolism was found, and the hypothesis is advanced that a lack of cholesterol in the blood serum may account for the increased hemolysis; and the splenomegaly may play some rô1e in the cause of this condition.

Urobilin and urobilinogen were present in the urine and feces, while bilirubin and hemoglobin were absent.