Pregnancy and cirrhosis of the liver

Pregnancy in patients with cirrhosis of the liver is uncommon. We reviewed 9 pregnancies in 7 patients with cirrhosis. One patient conceived within 1 month of diagnosis and in another the disease was diagnosed during the index pregnancy. Four patients has associated portal hypertension and 1 of them conceived after lienorenal shunt. Complications associated with these pregnancies were jaundice (1) jaundice plus ascites (2) and gastrointestinal bleeding (1). In 2 patients endoscopic sclerotherapy was done during the index pregnancy. The incidence of preterm delivery was 50% (4 of 8) and the majority (75%) occurred in pregnancies where associated complications were present. There was 1 maternal death in the postpartum period due to fulminant hepatic failure.     

Two successive pregnancies after ursodeoxycholic acid therapy in a previously infertile woman with antimitochondrial antibody-negative primary biliary cirrhosis

OBJECTIVE: To describe the benefit of ursodeoxycholic acid (UDCA) for the initiation and completion of a successful pregnancy in a previously infertile woman with primary biliary cirrhosis. DESIGN: Case report. SETTING: A university hospital with relevant departments. PATIENT(S): A 29-year-old woman with primary biliary cirrhosis and failure to conceive for 6 years. INTERVENTION(S): Establishment of diagnosis with a liver biopsy, pretreatment of patient with UDCA before conception, and continuation of UDCA after first trimester until term. UDCA was used in the second pregnancy again after the first trimester. MAIN OUTCOME MEASURE(S): Achievement of a safe conception and full-term pregnancy. RESULT(S): Two consecutive successful pregnancies, a healthy 3,250-g male infant and a healthy 3,000-g female infant. The second conception occurred in a period without the use of UDCA, implicating a latent beneficial effect of either UDCA or the previous pregnancy via some possible immune mechanism. CONCLUSION(S): Ursodeoxycholic acid could help achieve conception in infertile women with primary biliary cirrhosis. The use of UDCA after the first trimester is shown to be safe in two consecutive pregnancies. Although it cannot be conclusive, the unintentional use of UDCA in the first 20 days after conception did not result in any teratogenicity in the first child.     

Uncomplicated pregnancy in patients with unrecognized primary biliary cirrhosis]

A 32 yr-old woman suffering from an unsuspected primary biliary cirrhosis (PBC), completed an uneventual pregnancy. She only experienced diffuse pruritus and subicterus, which misled to an antepartum diagnosis of intrahepatic cholestasis of pregnancy. The most remarkable points of the reported case are: (a) the younger age of onset of PBC; (b) the paucisymptomatic course of PBC; (c) the rarity of PBC-associated pregnancy (only 15 previous instances in 12 patients, from a review of the literature); (d) the uncomplicated course of pregnancy for both the mother and the fetus, which is the exception rather than the rule, in such a condition. The present observation calls our attention to the possible existence of an underlying latent liver cirrhosis in pregnant women with pruritus, jaundice and mild alterations of liver function indexes. This association, which is expected to increase in frequency in the near future, may have important implications in the management of pregnancy.     

Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report.

BACKGROUND: Pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis.     

Pregnancy following the surgical treatment of portal hypertension

The course and results of 10 pregnancies in 7 patients who had undergone surgical treatment up to 9 years previously for portal hypertension are described. Four patients had extrahepatic portal hypertension, two had noncirrhotic intrahepatic portal hypertension, and one had frank biliary cirrhosis. Eight live babies were produced with no maternal deaths; the commonest complication was anemia during pregnancy, but only one patient bled from esophageal varices after shunting. Pregnancy after decompression operations and in extrahepatic obstruction is reviewed, and the particular hazards associated with pregnancy are discussed.     

Stricture of the common bile duct from chronic pancreatitis

Early diagnosis and treatment of stricture of the common bile duct from chronic pancreatitis are essential as the life-threatening complications of biliary cirrhosis and acute cholangitis can occur even in the absence of clinical jaundice. In a series of 40 patients with longstanding chronic pancreatitis and stricture of the common bile duct, findings included chronic pain in 26 patients, jaundice in 17 patients, secondary biliary cirrhosis in six patients and acute cholangitis in six patients. Persistent elevation of the alkaline phosphatase level was the most sensitive laboratory indicator of occult obstruction of the biliary tract. The diagnostic long tapered stenosis of the distal common bile duct was delineated by percutaneous transhepatic cholangiography in 21 of 22 patients and endoscopic retrograde cholangiopancreatography in eight of 11 patients. Operative decompression of the biliary tract included 32 biliary-enteric bypasses, three sphincteroplasty procedures and three emergency tube drainages. There was one postoperative death and seven other patients had postoperative complications. Sphincteroplasty failed to relieve the obstruction in two patients and two patients with biliary-enteric bypass had late anastomotic strictures develop which required reoperation. Only seven patients were free of pain at follow-up study which ranged from 0.5 to 15.0 years. Clinical suspicion based upon persistent hyperaklalinphosphatemia, diagnosis by cholangiography and decompression of the biliary tract by choledochoenterostomy can reliably avert the lethal complications of stricture of the common bile duct caused by chronic pancreatitis.     

Pregnancy in primary biliary cirrhosis.

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.     

Complications at Delivery in women with jaundice in pregnancy

As part of a follow-up of the women with jaundice in pregnancy reported by Thorling in 1955, maternal complications in 133 deliveries concerning 58 women were studied. Most of the 25 women who had itching and/or jaundice during several pregnancies probably suffered from recurrent cholestasis of pregnancy. The diagnoses in 24 women with jaundice during only one out of several pregnancies were infectious hepatitis and non-recurrent cholestasis of pregnancy. The remaining 9 women bore only one child each. Gestation tended to be shorter in pregnancies complicated by itching and/or jaundice at the time of delivery (median 38 weeks), than in pregnancies in which such symptoms had ended before delivery, or in uncomplicated ones (median 40 weeks). The duration of labour was not influenced by the presence of itching or jaundice. Such symptoms at the time of delivery did, however, appear to be associated with a higher incidence of excessive hemorrhage. The findings were similar in all groups of women, i.e. whether itching and/or jaundice had been recurrent or non-recurrent. The shorter gestation time and the tendency to larger hemorrhage were thus not primarily related to the condition causing itching and/or jaundice, but to the stage of pregnancy in which they occurred.     

Therapeutic effect of ursodeoxycholic acid on early-stage primary biliary cirrhosis.

Primary biliary cirrhosis is a rare chronic liver disease in Taiwan, which eventually causes mortality. As yet, no safe and effective treatment has been found. To investigate the safety and therapeutic efficacy of recently introduced ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis, an uncontrolled trial was conducted in 6 patients in the early stages (I-II) and 5 patients in the late stages (III-IV). Five patients in stage I and one patient in stage II were treated with 10-15 mg/kg/day UDCA for a mean administration period of 13 +/- 9 months. Levels of laboratory tests including serum alkaline phosphatase (ALP), alanine aminotransferase (ALT) and aspartate aminotransferase (AST) improved significantly within one month and were sustained at the new lower levels for the period of observation. The symptoms of one patient with pruritus were reduced after long-term therapy. No major side effects were found during the treatment period. In contrast to early-stage patients, patients with late-stage primary biliary cirrhosis who received UDCA therapy for a mean duration of 25 +/- 5 months showed no beneficial effects either clinically or biochemically. From these preliminary results, UDCA appears to be safe and effective in the treatment of early-stage primary biliary cirrhosis, although further controlled clinical trials in conjunction with histological follow-up are mandatory to evaluate the critical role of UDCA in primary biliary cirrhosis.     

Cholestatic jaundice of pregnancy: new perspectives

Cholestatic jaundice of pregnancy is a condition characterized by pruritus, icterus, or both during pregnancy. A total of 9 pregnancies complicated by cholestatic jaundice are presented. Three of these cases are presented in detail because they reflect complications that can occur with this condition. The literature on cholestatic jaundice is reviewed. This condition has been regarded in the past as benign to the mother and fetus, but these cases and other recent investigations suggest high perinatal mortality and morbidity rates.     

Autoimmune hepatitis and pregnancy: about a patient's three consecutive pregnancies

Spontaneous evolution of autoimmune hepatitis during pregnancy is not well known, nor are its consequences on pregnancy evolution. Immunosuppressive treatment during pregnancy is sometimes necessary but the long- and short-term consequences are unrecognized. We relate a patient's disease with autoimmune hepatitis and cirrhosis and three consecutive pregnancies with different obstetrical and medical complications.     

妊娠合并急性胰腺炎23例诊治分析

目的探讨妊娠期妇女并发急性胰腺炎的原因、临床早期诊断方法以及治疗措施。 方法回顾性分析2008年1月至2012年12月华中科技大学同济医学院附属同济医院收治的23例妊娠合并急性胰腺炎患者的临床资料,将其分为轻症组(16例)及重症组(7例),两组间计数资料比较采用Fisher确切概率法。 结果23例中合并胆道疾病8例(34.8%),高脂血症9例(39.1%);重症组血糖升高率达100%,较轻症组(12.5%)显著增高(P＝0.000),12例晚期妊娠分娩新生儿均存活,中期及晚期妊娠引产各1例,1例重症急性胰腺炎合并多器官功能衰竭患者母儿死亡,其余8例治愈出院继续妊娠。 结论妊娠合并急性胰腺炎的发生与胆囊疾病和高脂血症有关,早期诊断、个体化治疗、适时终止妊娠是提高治愈率的有效手段。     

Fatal disruption of a splenorenal shunt after multiple pregnancies

A 39-year-old woman underwent a distal splenorenal shunt operation for bleeding esophageal varices due to liver cirrhosis. Following the operation she had 7 pregnancies at almost yearly intervals. At the term of the last pregnancy a disruption of the anastomotic site caused a fatal hemorrhage. The outcome of postshunt pregnancies as reviewed in the literature is usually favorable, but numerous pregnancies in these circumstances may carry a considerable risk.     

Serum deoxycytidylate deaminase as an index of high-risk pregnancy

Summary. Serum deoxycytidylate deaminase (EC 3.5.4.12, dCMP deaminase) activity was routinely estimated over 7 years in 2460 pregnancies. The results confirm that elevated dCMP deaminase activity (>4.8 units) is associated with the development of pre-eclampsia but not with essential hypertension in pregnancy; a high rising level can precede eclampsia. Elevated enzyme activity was also found in women who developed jaundice in pregnancy and in some with apparently normal pregnancies. An unexpected finding was that these so called'false positive' high levels were associated with all the unexplained intrauterine fetal deaths that occurred later in pregnancy. Although elevated dCMP deaminase levels may be associated with a normal outcome, persisting high levels indicate the need for careful monitoring of fetal well being and, in the presence of any other sign of placental insufficiency, delivery should be expedited.     

Acute pancreatitis and pregnancy--analysis of a 10 year period of time

Acute pancreatitis during pregnancy is rare and complicates one in 1000 to 5000 pregnancies. The commonest reasons during pregnancy are: biliary disease, congenital or acquired hypertriglyceridemia and very rarely acute pancreatitis is associated with preeclampsia-eclampsia or HELLP syndrome. We presented in the research the clinical course of 14 women who developed acute pancreatitis and were treated at the Clinic of Thoraco-abdominal and Hepato-Biliary Surgery at the University Hospital Plovdiv for a tenth year of a period. The leading etiology was biliary disease. Eleven of 14 patient were successfully treated conservatively during pregnancy; 5 of them underwent surgery after pregnancy. Only one patient underwent operative treatment during pregnancy. Although acute pancreatitis is a rare disease during pregnancy, in order to improve the prognosis and to decrease the maternal and fetal morbidity and mortality, a timely diagnosis and an adequate conservative or surgical treatment is necessary.     

Liver diseases in pregnancy

Mild abnormalities of liver function tests are frequently seen in pregnancy but return to normal after delivery. A raised serum alkaline phosphatase is common, along with a decline in the serum albumin, but the aminotransferases remain within normal limits. The physician must interpret abnormal liver function tests in pregnancy with these changes in mind, but most liver diseases in pregnancy result in more marked alterations. Viral hepatitis is the most common cause of jaundice in pregnancy, and the maternal prognosis is generally good. Perinatal transmission of hepatitis B virus is likely when the mother is positive for HBsAg. Concurrent administration of hepatitis B vaccine and HBIG to the infant has an efficacy of 90 per cent in preventing transmission to the infant. ICP is the second most common cause of jaundice in pregnancy. The condition is generally benign, although maternal and fetal mortality occasionally result, probably due to premature delivery and the bleeding tendency of cholestatic patients. Vitamin K administration may correct the coagulopathy, and cholestyramine is effective in controlling pruritus. AFLP is rare but carries a high mortality rate for both the mother and the fetus. Early diagnosis, correction of the coagulopathy, and prompt delivery may improve the outcome significantly. Patients with cirrhosis have reduced fertility, and in those who become pregnant, fetal loss is high. The effect of pregnancy or hepatocellular function is variable, but, when evidence of liver failure is present in the first trimester, termination should be considered. Variceal size and the risk of bleeding may be assessed by endoscopy. Pregnant cirrhotic patients with large esophageal varices and a history of bleeding can undergo shunt surgery. Conservative management may be appropriate for patients with small varices and no history of bleeding.     

Surgical treatment of malformations of the uterus.

The reproductive history in 13 patients with uterine malformations was reviewed. Following plastic repair, there was a high incidence of pregnancy salvage. Of 19 pregnancies, 14 resulted in term deliveries, four in premature deliveries and one in abortion; there are 14 living children. The surgical repair in two patients with primary sterility was the contributing factor in achieving pregnancies with full term deliveries.     

Maternal smoking in relation to the incidence of early neonatal jaundice

A case-control study was carried out to investigate the association between maternal smoking during pregnancy and the risk of early neonatal jaundice. A total of 1,569 cases of neonatal jaundice (2.7% of all singleton births) were identified from the 1984 Washington State birth certificates as having a bilirubin level greater than 10 mg/dl within the first 2 days of life. 2,336 nonjaundiced infants were randomly selected to serve as controls. Information regarding smoking during pregnancy was also obtained from the birth certificates. After excluding infants with known risk factors for neonatal jaundice, 912 cases and 1,752 controls were available for analyses. Infants whose mother smoked cigarettes during pregnancy were at lower risk of neonatal jaundice (odds ratio = 0.81; 95% confidence interval = 0.66-0.99) relative to infants of nonsmokers. This relative risk changed very little after adjusting separately for maternal age, infant's gender, number of prior pregnancies, number of prenatal visits, marital status and number of prior fetal deaths. There was suggestive evidence that this apparent protective effect may be operating only among normal and high-birth-weight infants.     

Value of Maternal Anti-D Concentration in Predicting the Outcome of Rh(D) Haemolytic Disease

Summary: The outcomes of 43 pregnancies complicated by the presence of Rhesus antibodies were studied in relation to the peak concentrations of anti-D reached during pregnancy. Antibody concentration was measured by an automated method calibrated against the British Anti-D Working Standard. Where the anti-D concentration remained below 5 IU/ml, the infants at worst suffered only moderate jaundice controllable with phototherapy. Above this level the incidence of requirement for exchange or top-up transfusion was high with concentrations greater than 50 IU/ml being predictive of a very severely affected fetus. It is recommended that amniocentesis in these patients be deferred until maternal anti-D levels exceed 5 IU/ml.