S2705 Maternal and Clinical Outcomes in Pregnant Women With Acute Intermittent (Hepatic) Porphyria

Introduction: Acute intermittent porphyria (AIP) is a rare metabolic disorder characterized by life-threatening neurovisceral attacks, often triggered by hormonal fluctuations such as elevated progesterone levels. While the clinical spectrum and known triggers of AIP are well documented, pregnancy-related variations in comorbidities, healthcare utilization, and outcomes remain poorly understood. We aim to evaluate the impact of pregnancy on hospitalization characteristics and clinical outcomes among patients admitted with AIP. Methods: We utilized the National Inpatient Sample (2016-2020) to analyze woman AIP hospitalizations identified using International Classification of Diseases, Tenth Revision, Clinical Modification codes. Hospitalizations were stratified based on pregnancy status. Comparative analyses were conducted for baseline demographics, comorbidities, complications, and outcomes. Categorical variables were compared using χ² test and reported as percentage while continuous variables are presented as mean ± standard deviation. Results: We identified 125 hospitalizations of pregnant women with AIP, with a mean age of 28.3 years (60% were White and 16% were Black). Hypertension and obesity were the most common comorbidities. Sepsis/infection was the most frequent complication (16%), followed by acute kidney injury (10%) and venous thromboembolism (8%). No hepatic complications or in-hospital mortality were observed. Pregnant women had significantly higher hospitalization charges compared to non pregnant women ($96,698.63 vs $64,143.72; P = 0.0217). During the hospitalization, delivery occurred in 56% of pregnancies (64.3% were vaginal, with 77% being spontaneous). Preeclampsia and hyperemesis gravidarum were the most common pregnancy-related complications (12% each), followed by postpartum hemorrhage, fetal growth restriction, oligohydramnios/polyhydramnios, gestational hypertension, and medically induced abortion. No cases of preterm labor, intrauterine fetal demise, placental abruption, or spontaneous abortion were reported. Conclusion: Pregnant women with AIP experience notable maternal complications and incur significantly higher hospitalization charges despite the absence of hepatic complications or fetal loss. These findings highlight a critical knowledge gap and underscore the need for larger studies to better understand pregnancy-related outcomes and optimize care in this high-risk population.