ABSTRACT
Cystic fibrosis (CF) is the most common genetic disease in the United States (US) and, with the development of newer therapeutics, there is increased fertility among women with CF. We present a series of pregnant patients taking novel CF transmembrane conductance regulator (CFTR) modulators and summarize pertinent clinical considerations. All women conceived within four months after starting elexacaftor-ivacaftor-tezacaftor. Pulmonary function was stable before and during pregnancy. One patient developed transaminitis necessitating discontinuation of the medication mid-trimester. All patients delivered healthy neonates between 36-38 weeks of gestation with uncomplicated postpartum courses. No birth defects were encountered. Given that newly introduced CFTR modulators may increase fertility among CF patients, contraception counseling, pulmonary function monitoring, liver function monitoring, and multi-disciplinary care are important pillars of management.
ABSTRACT
Hepatic adenomas are benign liver tumors typically found in females of reproductive age. Though benign, hepatic adenomas are highly vascularized tumors, thus rupture and consequent hemorrhage present a feared complication. We report a case of a 31-year-old woman with hepatic adenoma who underwent preoperative portal vein embolization and subsequently suffered a rupture of her tumor. We postulate that the change in blood flow after portal vein embolization, a phenomenon known as the hepatic artery buffer response, may have contributed to the tumor rupture, though the possibility that the rupture was purely incidental remains. There is currently no prior report of such rupture occurring following portal vein embolization, and this case brings to light a potentially fatal complication of a generally safely regarded procedure in patients with hepatic adenoma.