Gupta AS
Liver disorders in pregnancy cause significant maternal and fetal morbidity and mortality. An obstetrician encounters 3 types of liver disorders while treating pregnant women. First are the incidental liver diseases that are caused by infective etiology like hepatitis A, B, C, E, Dengue hepatitis, and malaria. The second type of women are those already afflicted by chronic liver diseases or portal hypertension and have a coincidental pregnancy. There are five pregnancy related or pregnancy specific conditions of which three would require urgent intervention in the form of immediate delivery at the onset of the conditions. These five conditions are hyperemesis gravidarum that occurs in the first trimester, intra hepatic cholestasis of pregnancy, HELLP (hemolysis, elevated liver enzymes and low platelets), pre-eclamptic hepatic dysfunction, AFLP (acute fatty liver of pregnancy). The last three can prove catastrophic to the mother and child if the woman is not delivered immediately. I will be highlighting a few aspects of AFLP which has remained an under diagnosed condition.
AFLP is usually a condition of young primigravidas, in second half of their pregnancy who probably had a compensated hepatic mitochondrial fatty acid oxidation mechanism pre pregnancy. Decompensation of this process occurs in pregnancy probably due to the fetus being homo or heterozygous carrier of this autosomal recessive genetic disorder. Thus a compensated energy deficient woman decompensates when need for energy increases as in late pregnancy. Thus it can also be called as Mitochondrial hepatopathy. Prompt diagnosis and delivery can improve the maternal outcome as the placenta having the same genetic composition of the fetus increases the fatty acids (arachidonic acid) and its oxidative metabolites in the maternal circulation thus increasing load on the hepatic mitochondria.
Biochemical tests of liver are unaffected by pregnancy. Presence of jaundice, serum bilirubin levels usually very high or greater than 10 mg%, with aminotransferase levels moderately elevated, coagulopathy, abnormal serum creatinine levels, hypoglycemia (blood sugar less than 60 mg%), negative hepatic viral markers and blood smear negative for malarial parasite should make an obstetrician suspect AFLP. Clinical (Swansea) diagnostic criteria can be used to diagnose AFLP. Of the 14 parameters listed, presence of 6 in the absence of any other cause for liver failure are sufficient to aid early diagnosis of AFLP so as to start immediate, aggressive treatment (delivery). Abdominal pain, jaundice, vomiting, increased thirst or urination, encephalopathy, elevation of serum aminotransferases, creatinine, ammonia, white blood cell counts, uric acid levels, and prothrombin time; decreased blood sugar levels, presence of ascites/ bright liver on ultrasound and diagnostic liver biopsy showing diffuse or perivenular microvesicular steatosis are the 14 clinical parameters for diagnosing AFLP. These criteria have been validated against liver biopsy (gold standard). Liver biopsies are delayed due to coagulopathy. Prompt, early delivery after correction of coagulopathy can reduce maternal mortality to 10 % or less.
HELLP and pre-eclampstic liver dysfunction patients clinical presentation can overlap with AFLP and vice versa leading to difficulty in diagnosis. However, all three life threatening conditions have to be managed by urgent delivery. Multi disciplinary team approach, intensive care and urgent delivery can significantly improve obstetric outcomes.
An interesting care of pregnancy with in vitro fertilization appears in this issue who had hepatic dysfunction. The postulation of incipient pre pregnancy hepatic parenchymal insult due to ovarian hyperstimualtion leading to subsequent development of liver subcapsular hematoma makes for interesting reading.
AFLP is usually a condition of young primigravidas, in second half of their pregnancy who probably had a compensated hepatic mitochondrial fatty acid oxidation mechanism pre pregnancy. Decompensation of this process occurs in pregnancy probably due to the fetus being homo or heterozygous carrier of this autosomal recessive genetic disorder. Thus a compensated energy deficient woman decompensates when need for energy increases as in late pregnancy. Thus it can also be called as Mitochondrial hepatopathy. Prompt diagnosis and delivery can improve the maternal outcome as the placenta having the same genetic composition of the fetus increases the fatty acids (arachidonic acid) and its oxidative metabolites in the maternal circulation thus increasing load on the hepatic mitochondria.
Biochemical tests of liver are unaffected by pregnancy. Presence of jaundice, serum bilirubin levels usually very high or greater than 10 mg%, with aminotransferase levels moderately elevated, coagulopathy, abnormal serum creatinine levels, hypoglycemia (blood sugar less than 60 mg%), negative hepatic viral markers and blood smear negative for malarial parasite should make an obstetrician suspect AFLP. Clinical (Swansea) diagnostic criteria can be used to diagnose AFLP. Of the 14 parameters listed, presence of 6 in the absence of any other cause for liver failure are sufficient to aid early diagnosis of AFLP so as to start immediate, aggressive treatment (delivery). Abdominal pain, jaundice, vomiting, increased thirst or urination, encephalopathy, elevation of serum aminotransferases, creatinine, ammonia, white blood cell counts, uric acid levels, and prothrombin time; decreased blood sugar levels, presence of ascites/ bright liver on ultrasound and diagnostic liver biopsy showing diffuse or perivenular microvesicular steatosis are the 14 clinical parameters for diagnosing AFLP. These criteria have been validated against liver biopsy (gold standard). Liver biopsies are delayed due to coagulopathy. Prompt, early delivery after correction of coagulopathy can reduce maternal mortality to 10 % or less.
HELLP and pre-eclampstic liver dysfunction patients clinical presentation can overlap with AFLP and vice versa leading to difficulty in diagnosis. However, all three life threatening conditions have to be managed by urgent delivery. Multi disciplinary team approach, intensive care and urgent delivery can significantly improve obstetric outcomes.
An interesting care of pregnancy with in vitro fertilization appears in this issue who had hepatic dysfunction. The postulation of incipient pre pregnancy hepatic parenchymal insult due to ovarian hyperstimualtion leading to subsequent development of liver subcapsular hematoma makes for interesting reading.