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
ACUTE FATTY LIVER OF PREGNANCY
ReplyDeleteRare 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