Fróðskaparrit - 01.01.1969, Blaðsíða 10

18 Intermittent Intrahepatic Cholestasis of Unknown Etiology
Case 2 (EHR) born 1943. First attack 9 months old, for the following
7 years periods of jaundice of 2 to 3 months’ duration once to twice a
year. Laparotomy January 1950 showed macroscopically normal liver, and
the bile ducts appeared normal. No biopsy or cholangiogram was madc.
No attacks from the age of 7 to 15 years, since then regularly jaundiced
(fig. 1). During the free intervals no complaints except for food allergy.
Case 3 (HMT) born 1938. From the age of 1 to 6 years regularly
jaundiced, starting each December and lasting for about 6 months. At the
age of 22 years, while fishing in Greenland waters, a new episode of
jaundice, starting with slight arthralgia. Laparotomy March 1960 showed
a dark, but otherwise normal liver, normal extrahepatic bile ducts. No
biopsy or cholangiogram. Since then 3 episodes with a typical course,
except for slight arthralgia.
Case 4 (JPiD) born 1942. No jaundice or other significant disease
during infancy and childhood, the first episode of jaundice occurred at
the age of 19 years, laparotomy March 1961 showed normal peroperative
cholangiogram, macroscopically normal liver and pancreas. Since then 5
similar episodes and perfect well-being during the intervals.
Case 5 (JML) born 1941. Normal development during infancy and
childhood. The first episode of jaundice occurred at the age of 21 years,
while he was fishing in Greenland waters. Laparotomy January 1963
showed normal bile ducts at cholangiography, the liver looked normal
(biopsy was taken) and the pancreas was felt to be a little harder than
normal. During the following 3 years 4 more episodes of jaundice with a
similar course. Another laparotomy was performed October 1965, some
concrements could be felt in the gall bladder, but as it was without
inflammatory changes, and choledochotomy showed a normal common
duct, cholecystectomy was not performed. Due to recurrent attacks of
sharp abdominal pain and high urine amylase a third laparotomy was
performed at Rigshospitalet June 1966. The liver appeared normal, the
otherwise normal gall bladder contained 8 small, dark concrements, the
ductus choledochus was slightly dilated, and the cholangiogram revealed
a stenosis just below the entrance of the normal cystic duct, possibly
caused by the previous choledochotomy. The stenosis was reseoted. The
head of the pancreas was indurated and nodular, as in chronic in-
flammation; no biopsy of the pancreas was taken. Five days postopera-
tively cholascos developed, and a leakage at the site of resection had to
be repaired. The patient was well until one year after the operation, since
then he has had 3 episodes of relatively mild jaundice and moderately
severe abdominal pain.