Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY...

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Transcript of Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY...

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Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY Bahaa-El-Din Ewees MD Slide 2 Physiological changes in liver tests during normal pregnancy Test Normal Range Bilirubin Unchanged or slightly decrease AminotransferasesUnchanged Prothrombin time Unchanged Alkaline phosphatase Increases 2 to 4-fold Fibrinogen Increases 50% Globulin Increases in and globulins -fetoprotein Moderate rise, esp. with twins WBCIncreases CeruloplasminIncreases Cholesterol Increases 2-fold TriglyceridesIncreases Globulin Decreases in gamma-globulin Hemoglobin Decrease in later pregnancy Slide 3 Abnormal liver function tests occur in 3 - 5% of pregnancies for different reasons Liver diseases in pregnancy liver disorders that occur only in the setting of pregnancy liver disorders that occur only in the setting of pregnancy liver disorders that occur coincidentally with pregnancy liver disorders that occur coincidentally with pregnancy Slide 4 Liver diseases in pregnancy Only in the setting of pregnancy coincidental with pregnancy Preeclampsia- associated Chronic liver diseases e.g.: cholestatic liver disease, autoimmune hepatitis, Wilson disease, viral hepatitis, etc not associated with preeclampsia The preeclampsia itself HELLP-syndrome AFLP Hyperemesis gravidarum Intrahepatic cholestasis of pregnancy Slide 5 HELLP syndrome Slide 6 Severe preeclampsia is complicated in 2- 12% of cases (0.2-0.6% of all pregnancies) by hemolysis (H), elevated liver tests (EL), and low platelet count (LP), the HELLP syndrome. Severe preeclampsia is complicated in 2- 12% of cases (0.2-0.6% of all pregnancies) by hemolysis (H), elevated liver tests (EL), and low platelet count (LP), the HELLP syndrome. Etiology: microangiopathic hemolytic anemia + vascular endothelial injury fibrin deposition in blood vessels + platelet activation & consumption, small to diffuse areas of hemorrhage and necrosis large hematomas + capsular tears + intraperitoneal bleeding. Etiology: microangiopathic hemolytic anemia + vascular endothelial injury fibrin deposition in blood vessels + platelet activation & consumption, small to diffuse areas of hemorrhage and necrosis large hematomas + capsular tears + intraperitoneal bleeding. Slide 7 Clinical Features and Diagnosis Slide 8 Most patients: 27 - 36 weeks gestation, Most patients: 27 - 36 weeks gestation, but 25% in postpartum period. but 25% in postpartum period. Can occur with any parity and age but commoner in white, multiparous & older pts. Can occur with any parity and age but commoner in white, multiparous & older pts. Slide 9 Clinical Picture: Most patients Less commonly upper abd. pain & tenderness & tenderness Nauseavomiting Malaiseheadache Edema weight gain jaundice uncommon (5%) Hypertensionproteinuria renal failure + uric acid DI Antiphospholipidsyndrome some patients have no obvious preeclampsia Slide 10 Diagnosis requires the presence of all 3 laboratory criteria:Diagnosis requires the presence of all 3 laboratory criteria: Based on platelet count, may be: severe/ Class 1 (platelets 50,000), moderate/Class 2 (50 99,000), mild/Class 3 (100 150,000). Lately, DIC, pulmonary edema, placental abruption, and retinal detachment may be present. H HemolysisEL Elevated Liver Tests LP Low Platelets LDH>600 U/L indirect bilirubin AST> 70U/L Hospitalization & ICU care for: Hospitalization & ICU care for: o antepartum stabilization of BP and DIC, o seizure prophylaxis, o fetal monitoring. pregnancy is > 34 wk gestational age 24-34 wk immediate induction corticosteroids for 48 h (fetal lung maturity) delivery The only definitive treatment is delivery Slide 15 Corticosteroids which cross the placenta (betamethasone or dexamethasone,) for 24-48 hours fetal lung maturity improves maternal platelet count. Tried treatment modalities for patients with ongoing or newly developing symptoms Antithrombotics (Heparin, aspirin) plasmapheresis plasma exchange with FFP dialysis Slide 16 After delivery continue close monitoring of the mother Up to 48 h postpartum worsening thrombocytopenia & increasing LDH levels Most lab. values normalize After 48 h persistent or worsening lab. Abnormalities by 4 th postpartum day Postpartumcomplications May be normalization of platelets 5 days RARELY Slide 17 Fate & complications Slide 18 Reported maternal mortality is 1% Perinatal mortality rate ranges from 7%-22% and may be due to: Perinatal mortality rate ranges from 7%-22% and may be due to: premature detachment of placenta, premature detachment of placenta, intrauterine asphyxia, intrauterine asphyxia, prematurity. prematurity. Slide 19 Other complications : Other complications : No long-term effect on renal function noted. No long-term effect on renal function noted. abruptio placentae abruptio placentae DIC DIC ARF ARF ARDS ARDS pulmonary edema pulmonary edema stroke stroke liver failure liver failure hepatic infarction hepatic infarction Slide 20 Recurrence : Subsequent pregnancies carry a high risk of complications Recurrence : Subsequent pregnancies carry a high risk of complications pre-eclampsia, pre-eclampsia, recurrence, recurrence, prematurity, prematurity, IUGR, IUGR, abruptio placentae, abruptio placentae, perinatal mortality. perinatal mortality. Slide 21 Acute fatty liver Slide 22 Acute fatty liver of pregnancy (AFLP) is a rare but serious maternal illness that occurs in the third trimester of pregnancy. Acute fatty liver of pregnancy (AFLP) is a rare but serious maternal illness that occurs in the third trimester of pregnancy. Incidence: 1/10 000 to 1/15 000 pregnancies. Incidence: 1/10 000 to 1/15 000 pregnancies. Maternal mortality: 18% Maternal mortality: 18% Fetal mortality: 23%. Fetal mortality: 23%. More common in nulliparous women and with multiple gestation. More common in nulliparous women and with multiple gestation. Slide 23 Pathophysiology Defects in intramitochondrial fatty acid beta- oxidation (enzymatic mutations in fatty acid oxidation). Defects in intramitochondrial fatty acid beta- oxidation (enzymatic mutations in fatty acid oxidation). Heterozygous woman gets a homozygous fetus fetal fatty acids accumulate Heterozygous woman gets a homozygous fetus fetal fatty acids accumulate return to the mothers circulation return to the mothers circulation extra load of long-chain fatty acids extra load of long-chain fatty acids triglyceride accumulation hepatic fat deposition & impaired maternal hepatic function. triglyceride accumulation hepatic fat deposition & impaired maternal hepatic function. Slide 24 Clinical Features and Diagnosis Slide 25 Typical presentation: a 1 - 2 wk history of nausea, vomiting, abdominal pain & fatigue, a 1 - 2 wk history of nausea, vomiting, abdominal pain & fatigue, Jaundice (frequent), Jaundice (frequent), moderate to severe hypoglycemia, moderate to severe hypoglycemia, hepatic encephalopathy, hepatic encephalopathy, coagulopathy. coagulopathy. Slide 26 Laboratory findings aminotransferase levels (from mild elevation to 1000 IU/L, usually 300 - 500). aminotransferase levels (from mild elevation to 1000 IU/L, usually 300 - 500). Bilirubin: frequently > 5mg/dL. Bilirubin: frequently > 5mg/dL. Commonly: leukocytosis, anemia. Commonly: leukocytosis, anemia. With progress: thrombocytopenia ( DIC) & hypoalbuminemia. With progress: thrombocytopenia ( DIC) & hypoalbuminemia. May be: rising uric acid, renal impairment, metabolic acidosis, ammonia & biochemical pancreatitis. May be: rising uric acid, renal impairment, metabolic acidosis, ammonia & biochemical pancreatitis. Slide 27 Laboratory findings (Cont.) liver biopsy most definitive test often not done d. t. coagulopathy swollen, pale hepatocytes in the central zones microvesicular fatty infiltration (frozen section with oil red staining) findings Imaging studies (US & CT) Inconsistent So, diagnosis is usually based on clinical & lab. findings Slide 28 (B) Hematoxylin-eosin stain (high power) shows hepatocytes stuffed with microvesicular fat (free fatty acids) and centrally located nuclei. Histological appearance of the liver in AFLP Histological appearance of the liver in AFLP. (A) Sudan stain (low power) shows diffuse fatty infiltration (red staining) involving predominantly zone 3, with relative sparing of periportal areas. Slide 29 Treatment Slide 30 If no obstetric indication, normal delivery is preferred to CS ( % of major intra-abdominal bleeding) Careful attention to the infant: risk of cardiomyopathy, neuropathy, myopathy, nonketotic hypoglycemia, hepatic failure, and death. Treatment involves early recognition & diagnosis immediate termination of pregnancy + Slide 31 Fate & complications Slide 32 Usually By 2 - 3 days postpartum liver enzymes & encephalopathy improve Sometimes laboratory abnormalities persist after delivery & may initially worsen during first postpartum week Rarely patients progress to fulminant hepatic failure with need for liver transplantation. Most patients improve in 1 to 4 weeks postpart Slide 33 With advances in supportive management, the maternal mortality is now 7%-18% and fetal mortality 9%-23%. With advances in supportive management, the maternal mortality is now 7%-18% and fetal mortality 9%-23%. Complications: Complications: Infectious and bleeding remain the most life threatening. Infectious and bleeding remain the most life threatening. Liver transplantation has a very limited role because of the great potential for recovery with delivery. Liver transplantation has a very limited role because of the great potential for recovery with delivery. Slide 34 HOW TO DIFFERENTIATE Slide 35 HELLPAFLP % Pregnancies 0.2%0.6%0.005%0.01% Onset/trimester 3 or postpartum Family history NoOccasionally Presence of preeclampsia Yes50% Typical clinical features Hemolysis (anemia) Thrombocytopenia (50,000 often) Liver fa