Journal List > Korean J Obstet Gynecol > v.55(9) > 1088511

Han, Seo, and Im: Acute fatty liver of pregnancy with fetal microvesicular hepatic steatosis

Abstract

Acute fatty liver of pregnancy (AFLP) is a serious maternal disease occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Until recently the pathogenesis of AFLP was unknown and still has not been fully elucidated. However, recent molecular advances suggest that AFLP may result from mitochondrial dysfunction. Several reports have documented a strong association between AFLP and a deficiency of the enzyme long-chain 3-hydroxyacyl-CoA dehydrogenase in the fetus, a disorder of mitochondrial fatty acid beta-oxidation. Therefore in this case, through findings of liver biopsy from dead fetus, we report possible causal relationship between fetal liver disease and maternal AFLP with literature reviews.

Figures and Tables

Fig. 1
Microscopic findings of liver biopsy of dead fetus (Gram stain). (A) Low-power view (×100). (B) High-power view (×200). There are diffuse parenchymal hemorrhage with focal hemorrhagic infarction and microvesicular fatty metamorphosis (steatosis).
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Fig. 2
Hypothesis illustrating the possible role of fetal and maternal MTP mutations in developing AFLP. Carrying an LCHAD deficient fetus (A) is the major determining factor in the development of maternal illness. Hepatotoxic metabolites produced by the fetus and/or placenta may cause liver disease in the obligate heterozygous mother when combined with the metabolic stress of the third trimester. Environmental stress (B) may lead to the further accumulation of toxic metabolites in the genetically susceptible mother causing maternal liver disease. LCHAD, long-chain 3-hydroxyacyl-CoA dehydrogenase; AFLP,acute fatty liver of pregnancy; MTP, mitochondrial trifunctional protein. (From Ibdah. World J Gastroenterol 2006;12:7397-404 [15]).
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Table 1
Serial laboratory results
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HD, hospital day; PPD, postpartum day; AST, aspartate transaminase; ALT, alanine transaminase; LDH, lactate dehydrogenase; ALP, alkaline phosphatase; PTT, partial thromboplastin time; PT, prothrombin time; INR, international normalized ratio; WBC-Hb-Plt, white blood cell-hemoglobin-platelet; FDP, fibrinogen degradation product; r-GTP, gamma-glutamyl transpeptidase; CPK, creatine phosphokinase.

References

1. Knox TA, Olans LB. Liver disease in pregnancy. N Engl J Med. 1996. 335:569–576.
2. Treem WR. Mitochondrial fatty acid oxidation and acute fatty liver of pregnancy. Semin Gastrointest Dis. 2002. 13:55–66.
3. Buytaert IM, Elewaut GP, Van Kets HE. Early occurrence of acute fatty liver in pregnancy. Am J Gastroenterol. 1996. 91:603–604.
4. Reyes H, Sandoval L, Wainstein A, Ribalta J, Donoso S, Smok G, et al. Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients. Gut. 1994. 35:101–106.
5. Holzbach RT. Acute fatty liver of pregnancy with disseminated intravascular coagulation. Obstet Gynecol. 1974. 43:740–744.
6. Burroughs AK, Seong NH, Dojcinov DM, Scheuer PJ, Sherlock SV. Idiopathic acute fatty liver of pregnancy in 12 patients. Q J Med. 1982. 51:481–497.
7. Castro MA, Ouzounian JG, Colletti PM, Shaw KJ, Stein SM, Goodwin TM. Radiologic studies in acute fatty liver of pregnancy. A review of the literature and 19 new cases. J Reprod Med. 1996. 41:839–843.
8. Kamijo T, Wanders RJ, Saudubray JM, Aoyama T, Komiyama A, Hashimoto T. Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients. J Clin Invest. 1994. 93:1740–1747.
9. Ushikubo S, Aoyama T, Kamijo T, Wanders RJ, Rinaldo P, Vockley J, et al. Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits. Am J Hum Genet. 1996. 58:979–988.
10. Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med. 1999. 340:1723–1731.
11. Rinaldo P, Raymond K, al-Odaib A, Bennett MJ. Clinical and biochemical features of fatty acid oxidation disorders. Curr Opin Pediatr. 1998. 10:615–621.
12. Pons R, Roig M, Riudor E, Ribes A, Briones P, Ortigosa L, et al. The clinical spectrum of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr Neurol. 1996. 14:236–243.
13. Yang Z, Zhao Y, Bennett MJ, Strauss AW, Ibdah JA. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. Am J Obstet Gynecol. 2002. 187:715–720.
14. Yang Z, Yamada J, Zhao Y, Strauss AW, Ibdah JA. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA. 2002. 288:2163–2166.
15. Ibdah JA. Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications. World J Gastroenterol. 2006. 12:7397–7404.
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