Curriculum for Specialty Certificate Examination in Gastroenterology

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Thursday, 13 March 2014

Question Liver and pregnancy

A 29 yrs old woman who was 32 weeks pregnant presented to A & E with 2 weeks history of malaise, nausea and vomiting.
O/E there was no dtigmata of CLD, pulse 100 bpm, BP 160/94. She had right upper quadrant tenderness and prepheral odema.

investigatons:
Hb 110
Plt 68
INR 1.7
blood film schistocytes, spherocytes

Bili 74
ALT 176
AST 260
ALT 230
LDH 720

what is the most likely diagnosis?
1-acute fatty liver of pregnancy
2-Budd-Chiari
3-HELLP
4-Hepatits E
5-Intrahepatic cholestasis of pregnancy

1 comment:

  1. ACUTE FATTY LIVER OF PREGNANCY
    Rare 1:7000-13000, potentially fatalThird trimester, ( 30-38 weeks).1st and multiple pregnanciesMaternal mortality up to 10-20%Fetal mortality up to 20-50%

    Pathogenseis:
    Metabolic disorder / Mitochondrial dysfunction
    LCHAD is an important beta oxidation enzyme
    Breaks down L-C Fatty acids in liver
    Provides major energy source in muscle
    Is essential for intermediary metabolism in liver
    Severe maternal illness in pregnancy: AFLP
    Sudden death in early childhood with hypoglycaemia
    Hormones may affect mitochondrial function

    Clinical features:
    Vague symptoms
    –Nausea & vomiting
    –Malaise
    –RUQ pain 50-80%
    Mild features of pre-eclampsia
    Jaundice after 1-2 weeks
    Hypoglycaemia, clotting abnormalities
    Untreated leads to fulminant hepatic failure

    Diagnosis and treatment:
    Lab tests:
    –Elevated ALT and Bilirubin
    –DIC, Coagulopathy, Elevated ammonia
    –Renal failure
    -Uric acid commonly high
    Imaging:
    –To exclude other diagnoses
    –Biopsy is diagnostic
    Treatment:
    –Maternal stabilisation
    –Emergency delivery

    Intrahepatic cholestasis of pregnancy:

    Serious and common complication
    Geographical variation
    Fmailial condition 0.1-1.5% of european pregnancies
    Fetal distress in up to 33%
    Prematurity in up to 60%
    Intrauterine death 2% up to 20% untreated)
    Arryhthmia is cause of fetal death

    Pathogenesis
    Genetic susceptibility: heterozygote MDR3
    Hormonal: sex hormones can modify MDR3 gene expression and impair function of transport systems
    Increased gut permeability to endotoxin
    Increased bile acids act on endometrium and fetal cardiac muscle

    Clinical features
    2nd/ 3rd trimesters(>26 weeks)
    Pruritus (palms and soles)
    Jaundice uncommon 25%
    Raised ALT 60% Gamma GT 30%
    Raised serum bile acids
    Recurs in subsequent pregnancies in 40-60%
    Imaging of liver appears normal

    Management
    Close monitoring from diagnosis
    Delivery by 37-38 weeks
    Treat with UDCS and/or dexamethasone
    Vitamin K prevent post-partum bleed
    HELLP syndrome:

    Complication of severe pre-eclampsia
    4-20% of eclamptic pregnancies
    Occur in 1 to 6 in 100 pregnancies and between 16 weeks gestation and 3rd day postpartum.

    Haemolysis (elevated LDH)
    ELevated liver enzymes (ALT x 2-10)
    Low Platelets (<100 data-blogger-escaped-font="">
    Clinical presentation
    Third trimester 2/3, post partum 1/3
    Nausea, vomiting, malaise, headache
    RUQ pain, tender hepatomegaly
    Mortality: maternal 1%, fetal 35%
    Clinical presentation
    Third trimester 2/3, post partum 1/3
    Nausea, vomiting, malaise, headache
    RUQ pain, tender hepatomegaly
    Mortality: maternal 1%, fetal 35%
    –DIC
    –Placental rupture
    –Renal failure
    –Pulmonary oedema
    –Hepatic haematoma

    Diagnosis
    Laboratory tests
    –FBC, with peripheral smear (haemolysis)
    –Low platelets (<100 data-blogger-escaped-br="">–LDH >600
    –ALT >100
    –Uric acid >7.8
    –Creatinine >100
    –Proteinuria

    Imaging
    –CT/MR useful -hepatic infarction or haematoma
    Treatment:
    Urgent hospitalsation, magnesium, antihypertensive, antiplatlets
    Delivary is the only defentive treatment for preterm cases

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