1047 Congenital Analbuminemia: A Saskatchewan Case Series

Background: Congenital analbuminemia (CAA) is characterized by the absence of serum albumin. Typically albumin is the most abundant protein in the circulatory system and provides 80% of the colloid osmotic pressure. Albumin is responsible for carrying many molecules through the circulation. Therefore it unexpected that survival without albumin is possible. The worldwide prevalence of CAA is estimated at 1 in 1,000,000. There are 43 cases of CAA reported internationally, but there is very little information known about the impact of the absence of albumin. We have identified a population in Saskatchewan with a high incidence of CAA. Our intent is to review these cases and determine whether CAA has an impact on these patient morbidity and/or mortality. Methods: We identified thirteen cases of CAA from health records. Data collection included antenatal/obstetrical history, birth history, placental pathology, frequency of respiratory tract infections, frequency of hospitalization, complications during hospitalizations, co-morbid conditions, laboratory investigations, clinical signs/symptoms suggestive of analbuminemia and cause of death (if applicable). Results: CAA may predispose to preterm delivery, small for gestation infants and/or hydropic placentas. The cases had frequent admissions to hospital, primarily for respiratory tract infections. There were a high number of admissions to intensive care. Conclusion: Our case series indicates there is an increase morbidity and possibly mortality associated with CAA. We are not able to make any conclusions on the long-term risks of CAA. This review provides support for a longitudinal study of this population of patients.