General information
Liver disease in pregnancy
Description
This theme will focus on data acquisition and development of guidelines for management of rare gestational liver diseases (acute fatty liver of pregnancy; HELLP syndrome; severe, early-onset intrahepatic cholestasis of pregnancy) and pregnancy in women with rare liver diseases (cirrhosis with portal hypertension, autoimmune and genetic liver disease, vascular liver disease and non-cirrhotic hypertension, rare tumours, post-transplant).
Genetics
Guidelines will be developed for when it is appropriate to do genetic screening for gestational liver disease.
AFLP: offspring samples should be screened for disorders of fatty acid oxidation. This can be done by biochemical screen (acyl carnitines) or genetic screen. Severe, early-onset ICP: cholestasis panel, or ABCB4 and ABCB11 screening. Portal vein thrombosis: screen for hereditary or acquired thrombophilia.
At present there is insufficient data to justify genetic screening for most other gestational liver diseases.
Clinical presentation
For gestational liver disease: AFLP: acute hepatic failure in the 3rd trimester with no other cause identified, include 6 Swansea criteria. Severe, early-onset intrahepatic cholestasis of pregnancy: onset of pruritus before 33 weeks’ gestation, maternal serum bile acids ≥40µmol/L. HELLP syndrome: clinical features of pre-eclampsia (hypertension after 20 weeks’ gestation in conjunction with either proteinuria, feto-placental dysfunction and other end organ damage (liver impairment, renal insufficiency, neurological or haematological complications) in addition to diagnostic laboratory results.
Risk factors
Pregnancy. The theme is of relevance to women of reproductive age.
A family history of the gestational liver disease will indicate increased risk.
Symptoms
See clinical presentation
Diagnosis
For pre-existing liver disease, the diagnosis will have been made and women will have a positive pregnancy test
Management
AFLP: stabilise coagulopathy, hypoglycaemia, consider N-acetyl cysteine and admit to HDU or ITU. Severe, early-onset ICP: consider UDCA, if serum bile acids ≥100µmol/L delivery by 36 week’ gestation. HELLP syndrome: treat hypertension, consider magnesium sulphate, stabilise coagulopathy and other acute biochemical derangements, early delivery.
Complications
AFLP: acute hepatic failure, fetal loss, maternal death, children should be screened for disorders of fatty acid oxidation. Severe, early-onset ICP: stillbirth, spontaneous preterm birth, subsequent risk of hepatobiliary disease. HELLP syndrome: end organ damage resulting in maternal morbidity, e.g. cerebrovascular accident, retinal detachment, acute hepatic failure, fetal loss, maternal death.
CPMS
If you wish to discuss a rare liver pregnancy case with experts from the ERN, you can upload cases to the CPMS.
The CPMS is essential for interaction between healthcare professionals and experts on clinical decision making, and is provided by the European Union to all ERNs. CPMS supports online multidisciplinary meetings (tele-boards) to discuss patients with diagnostic or therapeutic dilemmas in need of expert consultation. CPMS offers the opportunity to upload and share clinical data of patients and pictures such as histological slides or MRI images. Importantly, this is fully in line with European data protection law. It is obligatory to inform patients about CPMS and obtain their informed consent before their data are entered into the system (see CPMS Privacy Statement published by the European Commission on 14 December 2017). The consent form is available in all EU languages in CPMS.
Clinical practice guidelines
Please click here to view clinical practice guidelines for the diseases covered by ERN RARE-LIVER.