Fróðskaparrit - 01.01.1969, Blaðsíða 17

Intermittent Intrahepatia Cholestasis of Unknown Etiology 25
criteria mentioned have been reported, 18 males and 6 females.
Six further cases, 5 maies and 1 female, probably should be
included, viz. case 2 of de Silva et al.10 who only had 2 epi-
sodes of jaundice, 3 relatives to the case of Biempica (11, per-
sonal communication from dr. Arias), one relative to Goldberg’s
cases14, and the sister of case 3 of this series.
The term intrahepatic oholestasis implies obstruction to the
flow of bile within the liver. It is used when olinical and
biochemical data are interpreted as biliary obstruction and no
obstruction is found macroscopically. The lesion may be loca-
lized to the wall of the bile capillaries, i. e. in the liver cell
itself, or to the intra- and interlobular bile ducts. The usual
morphological examinations have not revealed the site of the
lesion in intermittent intrahepatic oholestasis, but by 3-dimen-
sional reconstruction of an interlobular bile duct in the case
described by Levy et al.°, obstruction by swollen cells, pro-
truding into the lurnen of the duct, were found.
The etiologic factor probably should be sought among the
following categories: infection, toxic agents, and metabolic fac-
tors. Due to the unequivocal signs of liver damage during the
episodes, several authors (3,6) favour the notion that it is a
type of virus hepatitis. The great number of relapses in most
of the patients, however, and the apparently complete restitu-
tion in the intervals is distinctly different from the usual
course of viral hepatitis, and there is no positive epidemio-
olgic evidence to support the hypothetis. The liver cell damage
can equally well be due to the effect of a toxin or to a meta-
bolic derangement, or even to cholestasis as such21.
An exogenic toxin as the etiologic agent could explain why
several cases, as those of the present series, occur in small
groups. In that case the toxin should be widely distributed,
however, because the reported cases come from many parts of
the world, viz. from the Faroe Islands, five from Great
Britain1,6, four from the United States5 7 8 13, two from Bel-
gium8, two from Germany4, two from Italy14, one from
France9, one from Japan10, one from Greece11, and one from
3 — Fróðskaparrit