Fróðskaparrit - 01.01.1969, Blaðsíða 11

Intermittent Intrahepatic Cholestasis o£ Unknown Etiology 19
Family histories.
None of the patients were closely related, but great-grandfathers of
case 1 and case 2 were brothers.
The occurrence of similar symptoms among the relatives of the patients
was only noted in one case. The sister of case 3, born in 1942, was severely
jaundiced for several months when she was 4 years old. She had been
complaining of abdominal pain and itching for some time before. She
was treaced by bed rest at home, and no tests were made. At the age of
17 she suffered from general malaise, itching and periodically pale stools
for aboút 6 months, followed by intense jaundice, pruritus and abdominal
discomfort for about 2 months. The icteric index was 108, alkaline phos-
phatases 25 KA units, and alanine transaminases were normal. No liver
biopsy or surgical intervention was performed, the jaundice and pruritus
disappeared rather rapidly, and a cholecystogram performed shortly after-
wards was normal. Since then she has had two normal pregnancies without
jaundice or pruritus. She has not taken contraceptive pills.
The mother of case 5 has been jaundiced twice, once with prolonged,
severe itching. Jaundice of pregnancy among the relatives of the patients
has not been recorded.
Clinical findings during episodes of jaundice.
The patients were not aware of precipitating faotors. As seen in fig. 1,
the episodes in some cases ocourred with some regularity, but no fixed
seasonal pattern can be recognized. The feeding habits of the patients
were unremarkable, and they did not take drugs of any kind. An episode
of jaundice usually starts with fatigue, loss of appetite, nausea, and
sometimes vomiting. Simultaneously or a few days later itching starts,
disturbing the sleep at night. Dark urine and pale stools follows shortly
afterwards, and then jaundice becomes apparent, first scleral, then univer-
sal. Constant pain centrally in the abdomen between the xiphoid process
and the umbilicus without irradiation often occurs during the first week
of an attack, and this is occasionally the initial symptom. In case 5 the
pain usually is intense, requiring repeated injections of strong analgetics,
and in case 2 it is mild or absent. In the remaining patients the pain is
moderate.
The fatigue and poor appetite persist during the episode, causing a
weight loss of several kilograms. Usually the first sign of remission is
the return of the appetite, then some colour appears in the stools, the
itching diminishes and often disappears while the jaundice still is quitc
rnarked.
During the free intervals the patients appear clinically healthy and
have no specific complaints, but still they may have difficulties in re-