Successful Second Delivery After Liver Transplantation for Niemann-Pick Disease: A Case Report

Abstract

Background: Niemann-Pick disease (NPD) is a common autosomal recessive metabolic disorder. Patients with NPD often suffer from liver damage, and liver transplantation is one of the main methods for treating end-stage liver disease. However, pregnancy and delivery management in patients with NPD remain a challenging issue. Case Summary: In 2010, the patient underwent "liver transplantation + splenic artery ligation" at the Beijing 301 Hospital due to "liver cirrhosis and splenomegaly". She took "1 tablet/day of prednisone" after the surgery until nearly a year after the surgery. In 2013, due to "abnormal liver function", she took "half a tablet of prednisone" for 1 month after the surgery and has been taking tacrolimus ever since, with a dosage of "1.0mg in the morning and 1.0mg in the evening". In 2016, she underwent cesarean section due to "full-term pregnancy after liver transplantation and thrombocytopenia" at our hospital. After the surgery, she experienced nausea and vomiting, which improved after receiving methylprednisolone sodium succinate and symptomatic treatment. Liver function was normal on reexamination, and she was discharged. On March 1, 2019, she underwent lower segment cesarean section under general anesthesia and gave birth to a male infant, who was without abnormalities, weighed 3500g, had a nuchal cord of one week, an Apgar score of 10 points, and a complete placenta and amniotic sac delivery. Her uterus was poorly contracted, and she was injected with 100μg carboprost tromethamine intravenously. The lower segment of the uterus was sutured with absorbable suture No. 1 due to poor contraction, and the uterus contracted well after the suture. The uterus was routinely sutured, and the operation was successful with stable blood pressure. After the surgery, the patient had good mental status, sleep quality, normal bowel movements, and grade I wound healing of the abdominal incision. Liver and kidney function were normal on reexamination, and she was discharged. The mother and newborn were followed up after discharge, and at the end of the follow-up period, the mother's physical condition was good, and the infant's growth and development were normal. Conclusion: Women of childbearing age who have received liver transplantation for NPD can successfully conceive and deliver, but such patients have higher requirements for pregnancy guidance, immunosuppressive drug use, follow-up monitoring, perinatal care, and prevention of complications.