European Journal of Obstetrics & Gynecology and Reproductive Biology, 45 (1992) 149-151 0 1992 Elsevier Science Publishers B.V. All rights reserved 002%2243/92/$05.00
Pregnancy in primary biliary cirrhosis Kok-Kien Wong a and Khean-Lee Goh b ’ Department of Obstetrics and Gynaecology and h Department of Medicine, Medical Faculty, Uniuersity of Malaya, Kuala Lumpur, Malaysia
A 34-year-old multigravid woman with symptomatic primary biliary cirrhosis (PBC) of the liver had a successful pregnancy. A healthy baby was born prematurely at 36 weeks of gestation. Six months prior to the conception of this pregnancy, stage III PBC had been diagnosed. Portal hypertension and liver cirrhosis had not developed. It is uncommon for pregnancy to occur in the presence of PBC. In the case presented, the outcome of pregnancy was good and the liver function had not been significantly affected by the pregnancy. Primary biliary cirrhosis PBC; Pregnancy
Introduction Pregnancy in symptomatic PBC is an uncommon occurrence as reported in the world literature [ll. We present this case which was encountered in our clinical practice. Case report A 34-year-old Chinese woman was referred to antenatal care during her fourth pregnancy. Six months prior to conception, she had presented with non-icteric pruritus, weight loss and lethargy of 1 year’s duration. Hepatomegaly was detected, and her liver function tests were abnormal (Table I). Histopathology of a needle liver biopsy showed that although there was preservation of normal lobular architecture, the portal tracts were widened by chronic inflammatory cells forming lymphoid follicles. There was destruction of bile duct epithelium but no granulomas were seen. Some portal tracts showed loss of bile ducts and in-
Correspondence tot Dr. K.K. Wang, Department
of Obstetrics & Gynaecology, University of Malaya, 59100 Kuala Lumpur, Malaysia.
creased fibrosis was noted. Copper stains showed 2 + positivity. The overall histopathological picture was one of chronic non-suppurative destructive cholangitis and periportal hepatitis with portal fibrosis. The histological appearances were compatible with a diagnosis of Stage III PBC. Serology demonstrated positive IgG antimitochondrial, antinuclear and antiparietal cell antibodies. The IgG antimitochondrial titre was detected at above 1: 10 dilution. A hepatic ultrasound scan did not reveal any ductual obstruction and gastroscopy showed no oesophageal varices. A bone-marrow puncture revealed only iron depletion. The treatment prescribed consisted of iron, vitamins A, D and K supplements. In her past obstetric history, the patient had delivered 3 children aged 8, 6 and 5 years, respectively. The first child weighed 3.2 kg and was delivered by Caesarean section because of cephalopelvic disproportion. The other 2 children weighed 2.7 kg each at birth and were delivered normally at full term. The onset of the symptoms of PBC was 4 years following the least childbirth. In the current pregnancy, she attended for antenatal booking at 10 weeks of gestation. There was mild pruritus, slight jaundice and hepatomegaly of 4 cm below the right costal margin. Her liver function tests were abnormal (Table I). Vitamins A, D and K supple-
Liver function tests At diagnosis
Total bilirubin (NR 3.4-24
10 weeks gestation
Five months postdelivery
Aspartate transaminase CAST, SGOT) (NR 7-40 IU/I)
Alanine transaminase (ALT, SGPT) (NR 4-54 W/I)
Alkaline phosphatase (NR 34-135 W/I)
Albumin (NR 37-51
ments together with iron and folic acid were prescribed. The pruritus increased towards the third trimester and pregnancy eczema appeared. She was treated with topical hydrocortisone cream and oral cholestyramine 4 g thrice daily. The jaundice did not worsen and there was no deterioration in her liver function as her pregnancy progressed. Her blood coagulation profile was normal. Fetal surveillance with serial symphysio-fundal height measurements and ultrasound scans demonstrated satisfactory fetal growth. Premature labour occurred spontaneously at 36 weeks of gestation with normal delivery of a live male baby with good Apgar scores and weighing 2.6 kg. There was no postpartum haemorrhage and her puerperium was uneventful. Postpartum tubal ligation was performed at her request. At review 5 months after delivery, the patient remained well although there was presence of mild jaundice, pruritus and hepatomegaly. The liver function tests remained abnormal (Table I). Vitamins A, D and K supplements were continued with the addition of ursodeoxycholic acid 500 mg daily. Discussion
Pregnancy in symptomatic PBC is uncommon because of amenorrhoea, reduced fertility and the late onset of disease from 35 to 60 years of age [1,2]. However there is an increasing number of reports of pregnancy occurring in PBC. Till 1989, some 11 preg-
nancies have been reported in the world literature [2-41. The age of onset of PBC ranged between 16 and 34 years, whilst that at which pregnancy occurred in PBC was between 20 to 41 years. Among these pregnancies, 6 were successful [2-41, 2 were stillbirths [l] and 3 aborted spontaneously [1,2]. Deterioration of liver function, all in the third trimester, occurred in 5 cases with 2 stillbirths  and 3 livebirths [3,4]. The relationship between pregnancy outcome and liver function did not appear to be consistent. This 34-year-old patient had symptomatic PBC of one and a half years duration before she became pregnant. A diagnosis of PBC was made based on the histopathological picture of a liver biopsy and serological evidence. During the pregnancy and after, the liver function showed no significant change. The state of liver function is however variable, with reports of deterioration [3,4] or even improvement 141 during pregnancy. The other maternal complications such as toxaemia, postpartum haemorrhage, portal hypertension and oesophageal variceal haemorrhage did not occur here. The fetal outcome was good despite PBC and premature birth at 36 weeks of gestation. The birthweight corresponded to the 50th percentile for male infants. It has been noted, however, that the survival of the fetus in PBC may possibly be affected by the presence of maternal cirrhosis where fetal mortality could be high .
An important differential diagnosis to be considered in pregnant patients with intractable pruritus and jaundice is intrahepatic cholestasis of pregnancy. This condition, however, resolves after pregnancy, whereas PBC would persist. A circulating IgG antimitochondrial antibody is also detected in over 95% of patients with PBC but only rarely in other forms of liver disease . An elevated titre of this antibody was detected in this patient. The management of this patient centred around maternal and fetal surveillance. Symptoms and signs of deteriorating liver function were sought for together with monitoring of liver function. Fat-soluble vitamins A, D and K supplements were prescribed for her biliary dysfunction. We attempted to reduce her pruritus and eczema with cholestyramine and steroid creams. Portocaval amastomosis both before and during pregnancy had been performed to prevent or to treat bleeding oesophageal varices . However, sclerotherapy has been advocated to be a safer and less traumatic procedure in the management of this potentially fatal complication during pregnancy . Maternal mortality in cirrhotic patients is attributed to liver failure or bleeding from oesophageal varices . The successful outcome of this pregnancy could be related to the
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