Fróðskaparrit - 01.01.1969, Page 16

24 Intermittent Intrahepatic Cholestasis of Unknown Etiology
During the episodes of jaundice numerous bile plugs are found in the
bile canaliculi, together with signs of moderate liver cell damage, such
as a few necroses, some multinucleated liver cell, and variable stainability
af the cytoplasm. The portal tracts show slight to moderate inflammatory
infiltration mostly with monucleated cells, but also a few neutrophil and
eosinophil granulocytes. Biopsies taken during the free intervals are essenti-
ally normal. A detailed description of the light- and electronmicroscopic-
picture will be given elsehwere.
The gall bladder and bile ducts fail to visualize by cholecystography
during the episodes, but they appear normal during the intervals, except
for the calculi found in case 5. The increase in urine amylase usually
correlates well with the abdominal pain. In case 5 pancreatic involvement
was also demonstrated radiologically by retroperitoneal calcifications.
Pancreas secretion studies, performed in case 1 (1965) and case 5 (1967),
did not reveal decreased pancreatic function.
The EGG’s were strikingly similar in all the patients, showing a nega-
tive T-wave in lead III. There were otherwise no symptoms or signs of
heart disease.
Therapy.
Treatment with adrenocortical steroids and cholestyramine was tried
in all patients, but due to the great spontaneous fluctuations of the
disease, and the impracticability of a controlled trial, is it difficult to
evaluate the effect. Neither treatment could reproducibly suppress or
prevent the episodes. It is the impression of the patients, however, that
steroids are without any effect, but that cholestyramine relieves the
pruritus and possibly shortens the duration of the jaundice.
Discussion.
The first one or two episodes of intermittent intrahepatic
cholestasis almost inevitably wili be misdiagnosed as extra-
hepatic biliary obstruction and lead to laparotomy. When the
bile ducts are found to be normal, and several similar episodes
supervene, few diagnostic possibilities other than intermittent
intrahepatic cholestasis will exist. The clinical picture of inter-
mittent intrahepatic cholestasis is so characteristic and the
diagnostic criteria so tangible that it is surprising that the
syndrome has not been described earlier. This indicates that
the syndrome is very rare or, less likely, has come into exi-
stence recently.
During the last 9 years 24 patients fulfilling the diagnostic