Intrahepatic cholestasis of pregnancy

AKA
  • Cholestasis of pregnancy
  • Obstetric cholestasis
  • Cholestatic jaundice of pregnancy
  • Pruritus/prurigo gravidarum
 
Mechanism
  • Due to elevated bile acids
  • Etiology
    • Mutation in genes ABCB4, ABCB11, ABCC2 (encode bile transporter proteins)
    • High levels of sex hormones during pregnancy lead to transporter capacity to secrete substrates is exceeded
    • Hepatitis C infection
 
Pregnancy timeline
  • Usually begins in the 3rd trimester
 
Clinical presentation
  • Skin
    • Generalized pruritus without primary lesions
    • Secondary lesions: excoriations, prurigo nodularis like lesions
    • Dramatic pruritus
    • Usually start on palms and soles
    • +/- Jaundice
  • Systemic
    • Steatorrhea,
    • Vitamin k deficiency → risk of intra or post partum hemorrhage
 
Risk of recurrence with future pregnancies
  • Often recurs in subsequent pregnancies (45-70%) and with oral contraceptive pills (probably affect liver metabolism)
    • Provavelmente porque persiste aquela mutação do canal dos ácidos biliares
 
Mother and fetal complications
  • Risk of premature labor, fetal complications (specially when levels >40micromol/L)
  • Maternal hemorrhage (due to vitamin K deficiency from liver effects)
 
Exams
  • Increased serum bile acids (>11micromol/L in pregnancy, normal in non-pregnant 0-6)
  • Alkaline fosphatase is normally increased in pregnancy due to placental origin (GGT normal or decreased)
  • Brb
  • PT
  • +/- gallstones in ultrasound
  • hepatitis serology
 
Treatment
  • ursodeoxycholic acid
    • 15mg/kg/dia or 1g/dia
  • vitamin K (se icterícia e PT prolongado)
  • early delivery if fetal distress or maternal complications
  • Not recommended: S-adenosylmethionine, dexamethasone, epomediol, silymarin, phenobarbital, activated charcoal
  • Colestiramina é contraindicada por reduzir a absorção de vitamina K