Download Acute Fatty Liver of Pregnancy

Acute Fatty Liver of
Pregnancy
Jonathan Healan and Niamh O’ Shiel
Patient
• 25 year old seen in Antenatal clinic 10 weeks
gestation.
• G2P1 (still birth 38w back in 2011)
• Previous pregnancy normal until week 38, patient
felt nauseous with abdominal pain and was admitted
to hospital. Very unwell.
• No fetal heart rate on CTG confirmed IUD
• Vaginal delivery.
Patient
Liver enzymes 
Leucocytes 
Uric acid 
• PMHx: Nil
• FHx: Nil
• Diagnosis Fatty liver of pregnancy, liver enzymes
began to normalise following delivery with no
lasting effect on patient.
Acute Fatty Liver of
Pregnancy (AFLP)
• Obstetric emergency; Maternal and fetal mortality
significantly raised, 18% and 23% respectively.
• Usually occurs in third trimester. Microvesicular
infiltration of hepatocytes.
• Fortunately it is a rare condition. UK study found
incidence of 5/100,000.
• Gold standard of diagnosis is liver biopsy, but
Swansea classification relies on signs and symptoms.
These changes usually revert
to normal within days to
weeks following delivery
without persistent hepatic
injury.
(Ch’ng et al.2002)
Pathophysiology
• Abnormality in mitochondrial
β oxidation is recognised as the
cause of this condition.
• Long-chain 3-hydroxyacyl-CoA
dehydrogenase (LCHAD)
catalyses the third step in the β
oxidation of fatty acids in
mitochondria.
(Joshi et al. 2010)
• If fetus is deficient in LCHAD toxic metabolites can accumulate and cause
damage to the mothers liver. The long chain fatty acids are deposited in the
mothers liver giving rise to the characteristic appearance of steatosis
histologically.
(Joshi et al. 2010)
Pathophysiology
• It is recessive and so it is thought
that under normal physiological
conditions the mother has normal
fatty acid oxidation
• If both parents are heterozygous, the fetus can inherit both mutations and
will be unable to oxidise long-chain fatty acids
• The free fatty acids remain unmetabolised and are returned to the mother’s
circulation
• This causes strain on the mother’s liver and ‘overwhelms any diminished
maternal hepatic enzymatic activity’
• (Hin Ko & Yoshida, 2004)
Pathophysiology
• Progressive lipid accumulation in hepatocytes
• Usually fat content of liver is 5%, can go up to 19% in
AFLP
• Fat accumulation plus ammonia production leads to
coagulopathy and hypoglycemia
• Liver can be small, soft and yellow
• Microvesicular fat can also infiltrate
kidney, brain and bone marrow
(McNulty 2004)
Management
• Get the baby out!
• Early diagnosis, quick delivery and intensive care
• Stablise mother– airway management, IV fluids, correct
hypoglycemia, electrolyte and coagulation abnormalities,
blood products
• Once stabilised, deliver baby – vaginal birth if possible,
but often c-section is necessary
• Postpartum plasma exchange in severe cases
(Moldenhauer, O’Brien, Barton & Sibai, 2004)
Complications
• Patients at high risk of bleeding due to coagulation
problems – blood transfusion and fluids may be
needed
• Risk of hypoglycaemia – glucose infusion may be
needed
• Pancreatitis can develop following renal and hepatic
dysfunction
Prognosis
• Mortality from AFLP – app 18% for mothers, 23% for
fetus - deaths usually secondary to sepsis, renal failure,
circulatory collapse, pancreatitis or GI bleeding
• Maternal outcomes – liver function tests can
continuously worsen for up to a week but then recover
• Fetal outcomes – there can be fetal distress in a clinically
stable mother – so fetal monitoring is essential
• Recurrence of AFLP can occur in further pregnancies
but is quite uncommon
(Hin Ko & Yoshida, 2004)
Patient
• Autosomal recessive condition. Recurrence of
AFLP estimated around 20% in general population.
• Greater risk in primiparous women.
• Surveillance of pregnancy paramount, especially in
third trimester.
• Deliver baby quickly if showing signs of AFLP.
Hopefully at viable gestation, as delivery is only
cure.
GUT 2002
Prospective study of liver dysfunction in pregnancy in
Southwest Wales
C L Ch’ng, M Morgan, I Hainsworth,JGC Kingham
•
LFT abnormalities were investigated in 4377 deliveries during the 15
month study period.
•
Liver dysfunction was seen in 3% of deliveries during the 15 month
prospective study.
•
Of the 3% only 5 cases of AFLP. Close medical and obstetric
collaboration ensued low mortality.
References
• Hin Ko, H. & Yoshida, E. (2006) Acute fatty liver of
pregnancy. Canadian Journal of Gastroenterology. 20: 25-30
• McNulty J. Acute fatty liver of pregnancy. In: Foley MR,
Strong TH, Garite TJ, editors. Obstetric Intensive Care
Manual. 2. New York: The McGraw-Hill Companies Inc;
2004. pp. 207–15.
• Moldenhauer, J.S., O’Brien, J.M., Barton, J.R., & Sibai,
B. (2004). Acute fatty liver of pregnancy associated with
pancreatitis: a life-threatening complication. American
Journal of Obstetrics and Gynaecology. 190:502-505
• Marsha F. Browning, Harvey L. Levy, Louise E. WilkinsHaug, Cecilia Larson and Vivian E. Shih. (2006) Fetal Fatty
Acid Oxidation Defects and Maternal Liver Disease in
Pregnancy, American college of obstetricians and gynaecologists.
1:107.
• Ch’ng C, Morgan M, Hainsworth I, Kingham J. (2002)
Prospective study of liver dysfunction in pregnancy in
Southwest Wales. GUT. 51:876–880.
• Joshi D, James A, Quaglia A, Westbrook R, Heneghan
M. (2010) Liver Disease in Pregnancy. The Lancet.
375:594-605
• Willacy H. (2011) Jaundice in Pregnancy. Patient.co.uk.
Available at: http://www.patient.co.uk/doctor/Jaundicein-Pregnancy.htm#ref-6