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Updated: May 12, 2026

Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Intrahepatic Cholestasis in a Pregnant Patient With Sickle Cell Disease: A Case Report
Yamini Sundara Priya Dasiah1, Martina Saeid1, Fatmaelzahraa Ahmed2
1Internal Medicine, New Medical Center (NMC) Royal Women's Hospital, Abu Dhabi, ARE.
Insights
Sickle cell intrahepatic cholestasis (SCIC) is a rare but serious complication in pregnant patients with sickle cell disease (SCD). Early diagnosis and management, including early delivery, are crucial for successful outcomes.
Area of Science:
- Obstetrics and Gynecology
- Hematology
- Hepatology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with various complications.
- Sickle cell intrahepatic cholestasis (SCIC) is a severe, potentially fatal SCD complication, primarily in homozygous SCD patients.
- Intrahepatic cholestasis of pregnancy (ICP) typically occurs in late pregnancy, marked by itching and elevated liver enzymes/bile acids.
Observation:
- A pregnant patient with homozygous SCD developed cholestasis at 25 weeks gestation.
- The patient presented with abnormal liver enzymes and high bile acid levels.
- Diagnosis of homozygous SCD was established during her second pregnancy.
Findings:
- Intrahepatic cholestasis in a pregnant patient with homozygous SCD is exceptionally rare.
- The patient required delivery at 30 weeks gestation due to rapidly worsening cholestasis.
- Successful management of SCIC in pregnancy was achieved.
Implications:
- This case highlights the importance of recognizing and managing SCIC in pregnant SCD patients.
- Prompt intervention, including preterm delivery, can lead to successful maternal and fetal outcomes.
- Further research into SCIC pathophysiology and management strategies in pregnancy is warranted.
Abstract:
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.
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