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Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY Bahaa-El-Din Ewees MD.

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Presentation on theme: "Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY Bahaa-El-Din Ewees MD."— Presentation transcript:

1 Acute fatty liver versus HELLP syndrome in obstetric ICU: why and how to differentiate? BY Bahaa-El-Din Ewees MD

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 α and ß globulins α -fetoprotein Moderate rise, esp. with twins WBCIncreases CeruloplasminIncreases Cholesterol Increases 2-fold TriglyceridesIncreases Globulin Decreases in gamma-globulin Hemoglobin Decrease in later pregnancy

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

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

5 HELLP syndrome

6 Severe preeclampsia is complicated in 2- 12% of cases ( % 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 ( % 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.

7 Clinical Features and Diagnosis

8 Most patients: weeks’ gestation, Most patients: 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.

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

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 <150,000

11 Aminotransferase: variable, from mild to 10 – 20 fold, Aminotransferase: variable, from mild to 10 – 20 fold, Bilirubin: usually < 5 mg/dL. Bilirubin: usually < 5 mg/dL. Liver CT: Liver CT: subcapsular hematomas, subcapsular hematomas, intra-parenchymal hemorrhage, or infarction intra-parenchymal hemorrhage, or infarction hepatic rupture. hepatic rupture. Histologically: focal hepatocyte necrosis, periportal hemorrhage, and fibrin deposits. Histologically: focal hepatocyte necrosis, periportal hemorrhage, and fibrin deposits.

12 CT abdomen of a woman with severe HELLP syndrome (39 weeks). A large subcapsular hematoma extends over the Lt lobe; Rt lobe has heterogeneous, hypodense appearance due to widespread necrosis, with “sparing” of the areas of lt lobe (compare perfusion with the normal spleen).

13 Treatment

14 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 wk immediate induction corticosteroids for 48 h (fetal lung maturity) delivery The only definitive treatment is delivery

15 Corticosteroids which cross the placenta (betamethasone or dexamethasone,) for 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

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

17 Fate & complications

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.

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

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.

21 Acute fatty liver

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/ to 1/ pregnancies. Incidence: 1/ to 1/ 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.

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 mother’s circulation return to the mother’s 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.

24 Clinical Features and Diagnosis

25 Typical presentation: a wk history of nausea, vomiting, abdominal pain & fatigue, a 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.

26 Laboratory findings aminotransferase levels (from mild elevation to 1000 IU/L, usually ). aminotransferase levels (from mild elevation to 1000 IU/L, usually ). 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.

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

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.

29 Treatment

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 +

31 Fate & complications

32 Usually By 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

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.

34 HOW TO DIFFERENTIATE

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 failure with coagulopathy, encephalopathy hypoglycemia, DIC Aminotransfer- ases Mild, but may be up to fold rise typical but variable

36 HELLPAFLP Bilirubin <5 mg/dL unless massive necrosis often >5 mg/dL, higher if severe Hepatic imaging Hepatic infarcts Hematomas, rupture Fatty infiltration HistologyPatchy/extensive necrosis, periportal hge, fibrin deposits Microvesicular fat in zone 3 Maternal mortality 1%–25%7%–18% Fetal/perinatal mortality 11%9%–23% Recurrence in subsequent Pregnancies4%–19% fatty acid oxidation defect 25% No fatty acid oxidation defect rare

37 WHY TO DIFFERENTIATE

38 Major Risks Infections & bleeding (most life threatening). Hypoglycemia AFLP HELLP Pancreatitis (develop after onset of hepatic & renal dysfunction need serial screening of serum lipase and amylase for several days after hepatic dysfunction) DIC ARF ARDS pulmonary edema stroke & seizures liver hges (most life-threatening)

39 Therapeutic Options AFLP HELLP FFP glucose Liver transplant (limited role) Antithrombotics: (heparin, antithrombin, low dose aspirin) Steroids: rapid clinical & lab. improvement Blood transfusion Early Late Plasmapheresis Liver transplant More definite role role

40 Follow-up Precautions: A deficiency in long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is thought to be associated with the development of AFLP. Under normal circumstances, an individual that is heterozygous for enzymatic mutations in fatty acid oxidation will not have abnormal fatty oxidation. Under normal circumstances, an individual that is heterozygous for enzymatic mutations in fatty acid oxidation will not have abnormal fatty oxidation.

41 Affected patients should be screened for defects in fatty acid oxidation as recurrence in subsequent children is 25%, and recurrence of AFLP in mothers is also possible. Presymptomatic diagnosis of FAOD with The application of tandem mass spectrometry to newborn screening is an effective way to identify most FAOD patients presymptomatically Presymptomatic diagnosis of FAOD with The application of tandem mass spectrometry to newborn screening is an effective way to identify most FAOD patients presymptomatically reduce morbidity and avoid mortality reduce morbidity and avoid mortality

42 Current management of pts with FAOD includes long-term dietary therapy of: Current management of pts with FAOD includes long-term dietary therapy of:  fasting avoidance,  low-fat/high-carbohydrate diet  restriction of long-chain fatty acid intake and substitution with medium-chain fatty acids. These dietary approaches appear promising in the short-term, but not the long-term outcome. These dietary approaches appear promising in the short-term, but not the long-term outcome.

43 In conclusion Important to diff. AFLP from HELLP Important to diff. AFLP from HELLP Diff. mainly based on lab. + imaging (CT- MRI) Diff. mainly based on lab. + imaging (CT- MRI) Diff. because AFLP needs: Diff. because AFLP needs: o Maternal follow-up for recurrence o Baby follow-up for FAOD needing dietary control o Next pregnancies for presymptomatic diagnosis

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