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Djibre A, Zohar S and Assy N Acute fatty liver of pregnancy
Djibre A, Zohar S and Assy N, 30 March 2010
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Acute fatty liver of pregnancy (AFLP) is a rare disease of the third trimester with an incidence of 5/100,000 maternities. It occurs more often in a first pregnancy, in multiple pregnancies, and in the male fetus [1-3].

The perinatal mortality rate is high (10-66 %) and the maternal mortality is significant (12.5%) [2,4]. The main mechanism is impaired fetal mitochondrial fatty acid oxidation due to a mutation in long-chain 3-hydroxyacyl-CoA dehydrogenase [1,5]. It is not clear whether medications or acute viral illness increase the risk for developing AFLP [6]. The treatment is safe delivery of the baby and intensive supportive care [1].

Case history

A 23-year-old woman was admitted at 34 weeks of gestation of her first pregnancy for abdominal pain and intra-uterine fetal death, with cholestatic jaundice and acute renal failure.

She underwent cesarean section and was transferred to the intensive care unit. She gradually improved after periods of post-surgical disseminated intravascular coagulation. The patient had no previous medical record and had a normal pregnancy up to the 34th week.

Investigations

Two weeks before admission, the patient had upper respiratory tract infection and received antibiotics with no improvement. The patient had recurrent vomiting and received intravenous metoclopramide for 5 days. Two days before admission, her condition deteriorated, she appeared confused and was referred to the Emergency Room. Fetal monitoring and Doppler U.S. revealed a dead fetus.

Blood tests showed leucocytosis 28600 with 73% neutrophils, thrombocytosis 556000, Hb 14.4 g/dL, normal blood smear, total bilirubin 7.0 mg %, direct bilirubin 4.0 mg%, AST 208 IU, hyponatremia 127, hypokalemia 2.9, acute renal failure with creatinin 2.6 mg%, BUN 57, hypoglycemia 53mg/dL, uric acid 20 mg/dl, and LDH 646 IU/mL.

After fetal extraction, the patient developed disseminated intravascular coagulation (DIC) and hypocalcaemia with D-Dimeres 3.8, fibrinogen 178, PTT Ratio 1.46; ionized calcium 3.9 mg/dL. The fetal pathology showed macro vesicular steatosis with congestion of internal viscera. No macroscopic malformation was observed.

Management

The main therapeutic intervention was rapid fetal extraction by cesarean section. The patient received supportive treatment in intensive care unit with gradual recovery of hepatic and renal function tests and resolution of DIC. The patient returned to a normal clinical condition and was discharged. On follow-up visits: the liver enzymes were normal and she appeared well.

Discussion

Acute fatty liver of pregnancy has the worst prognosis of all pregnancy-related liver disorders. It is difficult to distinguish it from HELLP (Hemolytic, Elevated Liver enzymes, Low Platelets) syndrome that has similar clinical and biochemical features but a better prognosis [7]

Our patient had vomiting for 5 days before admission. Her blood tests showed liver enzyme elevation with prominent intrahepatic cholestasis. There was no evidence of extra-hepatic bile duct dilatation on liver US.

The serology for Epstein-Barr virus, Cytomegalovirus, hepatitis B, C, and A were all negative. HIV antibody was negative. The investigation for autoimmune liver disease (anti-nuclear/ mitochondrial/ parietal / smooth-muscle antibody) was negative. There was no evidence of an active infectious disease.

The presence of vomiting with liver enzyme elevation, acute renal failure and high LDH serum levels could indicate HELLP syndrome [1]. The presence of jaundice, the absence of features of preeclampsia (hypertension and proteinuria), the absence of thrombocytopenia and hemolysis were against this diagnosis.

The presence of liver failure (jaundice) with coagulopathy, encephalopathy, intra-uterine fetal death in the third trimester of a first pregnancy, with acute renal failure, leucocytosis, hypoglycemia, DIC and severe hyperuricemia support more the diagnosis of acute fatty liver of pregnancy [8].

The diagnosis was confirmed by post-mortem histology of fetal liver that showed massive fatty infiltration (Fig. 1). Vomiting, jaundice and increase uric acid at the third trimester of pregnancy should prompt immediate evaluation for acute fatty liver of pregnancy.

Figure 1A Hematoxyllin-eosin staining

Figure 1B PAS staining
Figure 1 Massive macro vesicular fatty infiltration of fetal liver (post-mortem)

This article was first published on GastroHep.com on 30 March 2010.

Authors

Djibre A, Zohar S and Assy N ,
1. Liver Unit, Ziv Medical Center, Safed, Israel.
2. Gynecology Department, Ziv Medical Center, Safed, Israel
3. Rappaport Faculty of Medicine, Technion Institute, Haifa, Israel

Corresponding author

Dr N Assy
Liver Unit, Ziv Medical Center
Safed 13100, Israel
Ph +972 4682 8441/5
Fax +972 4682 8442
Email Assy.n@ziv.health.gov.il or Assy.nimer@gmail.com

References

  1. Hay, JE. Liver disease in pregnancy. Hepatology 2008; 47: 1067-76.

  2. Knight M, Nelson-Piercy C, Kurinczuk JJ et al. A prospective national study of acute fatty liver of pregnancy in the UK. Gut 2008; 57: 951-6.

  3. Davidson KM, Simpson LL, Knox TA, et al. Acute fatty liver of pregnancy in triplet gestation. Obstet Gynecol 1998; 91: 806-8.

  4. Mjahed K, Charra B, Hamoudi D, et al. Acute fatty liver of pregnancy. Arch Gynecol Obstet 2006; 274: 349-53.

  5. Ibdah JA, Benett MJ, Rinaldo P, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 1999; 340: 1723-31.

  6. Saygan-Karamusel B, Kizilkilic-Parlakgumus A, Deren O, et al. Acute fatty liver of pregnancy after aspirin intake. J Matern Fetal Neonatal Med 2004; 16: 65-6.

  7. Hepburn IS, Schade RR. Pregnancy-associated liver disorders. Dig Dis Sci 2008; 53: 2334-58.

  8. Hsiung R, Hasselmann M, Lutun P, et al. [Acute fatty liver of pregnancy. Diagnostic value of hyperuricemia in the pre-jaundice stage][In French]. J Gynecol Obstet Biol Reprod 1988; 17: 901-5.

 
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