Dasiah Yamini Sundara Priya, Saeid Martina, Ahmed Fatmaelzahraa
Internal Medicine, New Medical Center (NMC) Royal Women's Hospital, Abu Dhabi, ARE.
Obstetrics and Gynaecology, New Medical Center (NMC) Royal Women's Hospital, Abu Dhabi, ARE.
Cureus. 2024 May 19;16(5):e60611. doi: 10.7759/cureus.60611. eCollection 2024 May.
Sickle cell intrahepatic cholestasis (SCIC) is a potentially fatal complication of sickle cell disease (SCD) with a high mortality rate, observed mainly in patients with homozygous SCD. Intrahepatic cholestasis of pregnancy is a known complication in pregnancy and usually presents in the late second or third trimester with itching, elevated bile acids, and elevated liver enzymes. Intrahepatic cholestasis in a pregnant patient with homozygous SCD is a rare occurrence. We present the case of a patient who was diagnosed with homozygous SCD during her second pregnancy and developed cholestasis with abnormal levels of liver enzymes at 25 weeks gestation, requiring delivery at 30 weeks gestation due to very high bile acid and liver enzyme levels. The patient was successfully managed.
镰状细胞性肝内胆汁淤积症(SCIC)是镰状细胞病(SCD)的一种潜在致命并发症,死亡率很高,主要见于纯合子SCD患者。妊娠期肝内胆汁淤积症是妊娠期已知的一种并发症,通常在妊娠晚期的第二个或第三个月出现瘙痒、胆汁酸升高和肝酶升高。纯合子SCD孕妇发生肝内胆汁淤积症的情况罕见。我们报告一例患者,其在第二次妊娠期间被诊断为纯合子SCD,并在妊娠25周时出现胆汁淤积且肝酶水平异常,由于胆汁酸和肝酶水平极高,在妊娠30周时需要分娩。该患者得到了成功治疗。