Curriculum for Specialty Certificate Examination in Gastroenterology

Countdown to the Examination

Tuesday 20 November 2012

Liver diseases in pregnancy

1- Hyperemesis Gravidarum:
1-20/1000 in the 1st trimester, 50% hospitalized
25% have abnormal liver biochemistry
raised conjugated bilirubin, ALP x2 normal, ALT up to 200iu/l
aetiology unknown
? rapidly rising steroid level
complications related to repeated vomiting
outcome of pregnancy normal

2-per-eclampsia:
hypertension, protienurea, odema
5-7% of pregnancies
2nd and 3rd trimester
can lead to seizures, renal failure, coma and death
hepatic infarction and rupture occur rarely

3-HELLP syndrome:
complication of severe pre-eclampsia
4-20% eclamptic pregnancies
3rd trimester 2/3, postpartum 1/3
Haemolysis (elevated LDH)
ELevated liver enzymes (ALT x 2-10)
Low Platelets (<100 p="">presentation: N/V, malaise, headache, RUQ pain, hepatomegaly.
mortality: 1% maternal, 35% fetal
severe morbidity: DIC, placental rupture, renal failure, pul odema.
lab tests: uric acid >7.8, LDH >600, ALT>100, smear: haemolysis

4-AFLP acute fatty liver of pregnancy:
rare, 1:7000-13000, potentially fatal
3rd trimester, 1st and multiple pregnanies
Mild features of pre eclampsia
Jaundice after 1-2 weeks
hypoglycaemia, clotting abnormality
Untreated leads to fulminant hepatic failure
lab tests: elevated ALT and bilirubin, DIC, coagulopathy, elevated amonia, renal failure
maternal mortality up to 10-20%, fetal mortality 20-50%

5-obstetric cholestasis:
2nd and 3rd trimester (>26 weeks)
prurius (plams and soles)
juandice uncommon 25%
raised ALT 60%, gamma GT 30%
raised serum bile acids
imaging of the lliver normal
close monitoring, delivary by 37-38 weeks
treat with UDCA and/or dexamethsone, vit K prevent post-partum bleed

4 comments:

  1. A 32-year-old patient is referred with severe pruritus and mild jaundice. She is 24 weeks pregnant (late second trimester). She reports similar symptoms with a prior pregnancy. Laboratory tests show serum total bilirubin 2.3 mg/dL (normal 0.3-1.2 mg/dL); alkaline phosphatase 321 U/L (normal 36-92 U/L); ALT 48 U/L (normal 0-35 U/L); and AST 44 U/L (normal 0-35 U/L). Which of the following statements is true with regard to this condition?

    A. Ursodeoxycholic acid at a dose of 8-15 mg/Kg/day is appropriate.

    B. Measurement of serum bile acids is not useful.

    C. This condition commonly presents in the first trimester of pregnancy.

    D. Recurrence in subsequent pregnancies is rare.

    Explanation

    This patient likely has intrahepatic cholestasis of pregnancy, a condition that is most common in the second and third trimester of pregnancy. UDCA is an accepted therapy and measurement of bile acids has been used to make the diagnosis. Recurrence is common and both genetic and environmental factors have been implicated.

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  2. A 30-year-old primiparous woman in her 29th week of pregnancy presents with severe pruritus. Physical examination reveals several spider angiomata, palmar erythema, diffuse excoriations and trace pedal edema. Her laboratory tests reveal an alkaline phosphatase of 160 IU/L (normal 80-120), bilirubin 3.0 mg/dl (normal 0.2-1.1), an INR of 1.8 and elevated serum bile acids. Which is the next step in your management?

    A. Immediate delivery for presumed AFLOP

    B. Refer to a transplant center and prepare for delivery

    C. Give her Vitamin K and ursodeoxycholic acid, send her home, repeat labs and follow

    D. ERCP for treatment of presumed choledocholithiasis

    Explanation

    The patient has cholestasis of pregnancy. This typically occurs in the third trimester and recurs commonly in subsequent pregnancies. There is often a family history of similar problems during pregnancy. The INR is elevated because of vitamin K malabsorption in the setting of cholestasis. Her alkaline phosphatase is elevated, but this is common in pregnancy due to the contribution of placental AP. Urso (ursodeoxycholic acid) should be given because it improves fetal outcome in the setting of cholestasis of pregnancy. It may also ameliorate the pruritus.

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  3. A 28-year-old woman attended a maternity clinic when 37 weeks pregnant with her first child. She complained of upper abdominal pain, nausea and vomiting.

    On examination, she was drowsy. Her pulse was 105 beats per minute, and her blood pressure was180/125 mmHg. Urinalysis showed protein 3+.

    Investigations:

    haemoglobin 113 g/L (115–165)
    platelet count 164 × 109/L (150–400)
    international normalised ratio 1.1 (<1.4)

    serum total protein 65 g/L (61–76)
    serum albumin 32 g/L (37–49)
    serum total bilirubin 25 µmol/L (1–22)
    serum alanine aminotransferase 61 U/L (5–35)
    serum aspartate aminotransferase 63 U/L (1–31)
    serum alkaline phosphatase 183 U/L (45–105)
    What is the most appropriate treatment?

    Answers
    A: caesarean section
    B: intravenous hydrocortisone
    C: oral colestyramine
    D: oral prednisolone
    E: oral ursodeoxycholic acid
    Correct answer: A
    Explanation
    This patient has severe pre-eclampsia (hypertension and proteinuria) and needs delivery of the baby. Liver disease is a common association, usually in the form of the HELLP syndrome, acute fatty liver of pregnancy, subcapsular hepatic haematoma or hepatic rupture.

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  4. A 32-year-old female presents with pruritus and jaundice. She is 30 weeks gestation in her first pregnancy.
    Two weeks earlier she had been treated by the ENT surgeons after presenting to the Emergency department with intractable nose bleeds.
    Liver function tests reveal:
    ALT 72 U/L (5-40)
    Alkaline phosphatase 700 U/L (30-110)
    Bilirubin 80 µmol/L (1-18)
    Serum bile acids 100 times normal titre
    Which of the following statements is correct concerning this patient?
    1-ALP does not increase in a normal pregnancy
    2-Maternal hepatic blood flow does not increase in pregnancy
    3-Treatment options include IV N-acetyl cysteine
    4-Varices are diagnostic of liver disease in pregnancy
    5-Viral hepatitis is the likely diagnosis

    The diagnosis here is intrahepatic cholestasis which presents with markedly elevated serum bile acids (cholylglycine).

    It presents in the second or third trimester and usually the alkaline phosphatase (ALP) is 7-10 times normal with raised alanine transaminase (ALT), aspartate transaminase (AST) and bilirubin.

    Cardiac output and blood volume increase in pregnancy but hepatic blood flow does not.

    Treatment options include ursodeoxychloric acid, cholestyramine, phenobarbital and vitamin K to treat the coagulopathy.

    ALP rises in pregnancy but not to this extent. The placenta is the source of the raised ALP.

    Viral hepatitis is the commonest cause of jaundice in pregnancy but the elevated bile acids make this unlikely in this case.

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