Fróðskaparrit - 01.01.1969, Page 20

28 Intermittent Intrahepatic Cholestasis of Unknown Etiology
o£ the pancreas. Sknilar abdominal pain has been described in
other cases5 7 14, but generally in a milder form and without
other evidence of pancreatitis. The factor whioh causes the
cholestasis (lithocholic acid?) might also affect the pancreas,
in most cases, however, to a minor degree.
In some instances prednisone6 and cholestyramine8 14 appears
to have produced a rather dramatic improvement. The inter-
mittence of the symptoms make the evaluation of therapy
difficult, but the general impression is that these drugs are of
limited value in most of the patients. If abnormal bile acid
metabolism plays an etiologic role, however, the effect of bile
acid sequestring therapy29 must be further explored.
No cases have been followed for a lifetime, and the final
outcome is unknown. It appears, however, that the jaundice
may continue to recur even if the severity of the episodes and
the duration of the free intervals may vary. It is also the
impression that the recovery during the free intervals is com-
plete, but progression to biliary cirrhosis has ibeen suspected in
one case4 and it may be significant that the BSP storage and
the galactose elimination capacity in case 1 and the BSP Tmax
of all the patients examined in this series during free intervals
(case 1, 2, 4 and 5) was reduced. The extensive histochemical
and electron microscopic examinations made during a free
interval by Biempica and coworkers11 revealed only minor
residual ohanges, but in view of the rather substantial signs
of liver cell damage during the jaundice, the risk of progres-
sive changes cannot be ignored. When the prognosis is eva-
luated, it must also be taken into consideration that the pati-
ents mostly are unable to do any work during the episodes on
account of general symptoms and will be more or less incapa-
citated even in the intervals, unless they are very long, in part
perhaps due to the incertainty which the constant threat of
recurrent attacks imposes. Is is therefore questionable if the
word benign should be included in the designation of the synd-
rome1. From a taxonomic point of view this is superfluous,
since there is no known malignant counterpart from which
the syndrome must be distinguished.