Fetal Fatty Acid Oxidation Defects and Maternal Liver Disease in Pregnancy

Browning, Marsha F.; Levy, Harvey L.; Wilkins‐Haug, Louise; Larson, Cecilia A.; Shih, Vivian E.

doi:10.1097/01.aog.0000191297.47183.bd

Cited by 127 publications

(85 citation statements)

References 30 publications

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“…23,24 It carries significant perinatal and maternal mortality and requires early diagnosis and intervention to prevent maternal and fetal death (Table 3).…”

Section: Acute Fatty Liver Of Pregnancymentioning

confidence: 99%

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Liver disease in pregnancy

2008

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Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes, including coincidental liver disease (most commonly viral hepatitis or gallstones) and underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related and caused by 1 of the 5 liver diseases unique to the pregnant state: these fall into 2 main categories depending on their association with or without preeclampsia. The preeclampsiaassociated liver diseases are preeclampsia itself, the hemolysis (H), elevated liver tests (EL), and low platelet count (LP) (HELLP) syndrome, and acute fatty liver of pregnancy. Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy have no relationship to preeclampsia. Although still enigmatic, there have been recent interesting advances in understanding of these unique pregnancy-related liver diseases. Hyperemesis gravidarum is intractable, dehydrating vomiting in the first trimester of pregnancy; 50% of patients with this condition have liver dysfunction. Intrahepatic cholestasis of pregnancy is pruritus and elevated bile acids in the second half of pregnancy, accompanied by high levels of aminotransferases and mild jaundice. Maternal management is symptomatic with ursodeoxycholic acid; for the fetus, however, this is a high-risk pregnancy requiring close fetal monitoring and early delivery. Severe preeclampsia itself is the commonest cause of hepatic tenderness and liver dysfunction in pregnancy, and 2%-12% of cases are further complicated by hemolysis (H), elevated liver tests (EL), and low platelet count (LP)-the HELLP syndrome. Immediate delivery is the only definitive therapy, but many maternal complications can occur, including abruptio placentae, renal failure, subcapsular hematomas, and hepatic rupture. Acute fatty liver of pregnancy is a sudden catastrophic illness occurring almost exclusively in the third trimester; microvesicular fatty infiltration of hepatocytes causes acute liver failure with coagulopathy and encephalopathy. Early diagnosis and immediate delivery are essential for maternal and fetal survival. (HEPATOLOGY 2008;47:1067-1076.) M ost pregnant women are young and healthy, and physiological changes in pregnancy must not be mistaken for liver dysfunction (Table 1). Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes (Table 2). Although relatively uncommon, any liver disease can occur coincidentally in the pregnant patient and pregnancy may occur in a patient with underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related 1 and due to one of the 5 liver diseases unique to the pregnant state-hyperemesis gravidarum (HG), intrahepatic cholestasis of pregnancy (ICP), preeclampsia, the HELLP syndrome, and acute fatty liver of pregnancy (AFLP). These conditions are complications of pregnancy itself, and each has a characteristic timing in relation to the trimesters of pregnancy: HG in the first trimester, ICP in the second half of pregnancy, and the other 3 in the third trimes...

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“…23,24 It carries significant perinatal and maternal mortality and requires early diagnosis and intervention to prevent maternal and fetal death (Table 3).…”

Section: Acute Fatty Liver Of Pregnancymentioning

confidence: 99%

“…Unlike HELLP, 40%-50% of patients with AFLP are nulliparous, with an increased incidence in twin pregnancies. 23,24 AFLP occurs almost exclusively in the third trimester from 28 to 40 weeks, rarely in late second trimester. In a few patients, it presents as jaundice in the postpartum period.…”

Section: Acute Fatty Liver Of Pregnancymentioning

confidence: 99%

Liver disease in pregnancy

2008

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“…The patient was also counseled regarding the theoretical possibility of developing acute fatty liver of pregnancy and hemolysis, elevated liver enzymes and low platelets syndrome, as these pathologies have been seen in other FA b-oxidation disorders. 8,9 Her prenatal laboratories and fetal sonographic examination in midgestation were unremarkable. Maternal serum acylcarnitine profile (Figure 1), liver enzymes, urine organic acids and plasma CK levels were followed up the throughout pregnancy.…”

Section: Casementioning

confidence: 99%

“…However, these complex interactions can occasionally be deleterious as seen in women carrying fetuses with long-chain 3-hydroxy acyl-CoA dehydrogenase deficiencies bear an increased risk for hemolysis, elevated liver enzymes and low platelets syndrome and acute fatty liver of pregnancy. 9 It is hypothesized that this results from relative carnitine deficiency secondary to increased consumption and accumulation of harmful acylcarnitine intermediates from the developing fetus. 9 The need and frequency for attainment of metabolic profiling in b-oxidation defects during pregnancy is unknown.…”

Section: Vlcad Deficiency In Pregnancymentioning

confidence: 99%

“…9 It is hypothesized that this results from relative carnitine deficiency secondary to increased consumption and accumulation of harmful acylcarnitine intermediates from the developing fetus. 9 The need and frequency for attainment of metabolic profiling in b-oxidation defects during pregnancy is unknown. As rising acylcarnitine and urinary medium and long-chain dicarboxylic acid profiles generally are associated with acute metabolic decompensation, 2 we reasoned that interval follow-up of profiles could be used for the prediction and management of our patient, as it can change as pregnancy progresses.…”

Section: Vlcad Deficiency In Pregnancymentioning

confidence: 99%

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Clinical and biochemical improvement of very long-chain acyl-CoA dehydrogenase deficiency in pregnancy

et al. 2010

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Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an enzymatic defect of the fatty acid (FA) beta oxidation pathway. In catabolic states, such as labor and early postpartum period, patients are potentially prone to metabolic decompensation and subsequent rhabdomyolysis with increased risk for myoglobinuria and renal insufficiency. We report a 21-year-old primigravida with a previously characterized VLCAD deficiency, who experienced frequent and unprovoked episodes of rhabdomyolysis before pregnancy. As there was no published experience to guide her management, a detailed multidisciplinary care plan was established to minimize the potential morbidity. Although there is little known about the antenatal course of gravidae affected by VLCAD, we predicted that placental and fetal b-oxidation in an unaffected pregnancy may temporize or even improve maternal FA b-oxidation. Consistent with our prediction, we observed a significant clinical and biochemical improvement throughout her pregnancy, and she delivered vaginally with an uncomplicated postpartum course. We conclude that although VLCAD deficiency can present a therapeutic challenge during pregnancy, the beneficial placento-maternal metabolic interactions and the implementation of a proper peripartum management reassure a successful antenatal and perinatal outcome.

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