Fróðskaparrit - 01.01.1969, Blaðsíða 19

Intermittent Intrahepatia Cholestasis of Unknown Etiology 27
case 4 and 5 t)he disappearance of intravenously injected un-
conjugated bilirubin was greater than normal, and in case 5
a considerable amount of alkali-stable monoglucuronide was
demonstrated23. Excess formation of abnormal conjugates was
suggested to be the metabolic defect of the syndrome, but too
little is known about these factors in other types of ohole-
stasis to warrant any conclusions.
Elevated bile acid concentrations in the serum has been
demonstrated in several patients8 1114 and is presumeably che
cause of the severe pruritus in all the cases24, but detailed
fractionation, especially with regard to the concentration of
unconjugated lithocholic acid, have not been performed. Since
this acid is capable of producing intrahepatic cholestasis and
liver cell damage25, an abnormal formation or reabsorption of
lithocholic acid as the primary metabolic defect in the synd-
rome must be considered. This would even provide an ex-
planation for the intermittence of the jaundice, as the chole-
stasis might prevent the production of lithocholic acid in the
intestines for a period. The formation of gallstones in case 5
may also be related to abnormal bile acid metabolism26.
Clinically there are many similarities between intrahepatic
cholestasis of pregnancy and the present syndrome. Among the
6 female patients with intermittent intrahepatic cholestasis,
the relation between pregnancy and episodes of jaundice has
not been marked, since coincidence only occurred once in eaoh
of two patients7 13. In one patient an episode started late in a
pregnancy and culminated several months after its termi-
nation12. Intrahepatic cholestasis of pregnancy typically recurs
during al pregnancies, disappears rapidly after the delivery27,
and never occurs outside pregnancy except in some patients
when treated with contraceptive pills28. It is felt that the evi-
dence of a common etiology in both syndromes is meager.
The role of the pancreatitis in case 5 in the syndrome is
uncertain. Three more patients in the present series had elevated
urine amylase, and it is likely that the abdominal pain during
the initial phases of the episodes of jaundice is due to affection