Hereditary factor X deficiency represents an uncommon challenge in pregnancy. A 30-year-old primigravida affected by severe factor X deficiency was followed from 6 weeks of gestation until delivery. Factor X was provided prior to delivery for the first time in pregnancy via plasma exchange. The pregnancy and postpartum period were not complicated by bleeding episodes; therefore this approach was accompanied by lower cost and fewer side effects when compared with fresh-frozen plasma and prothrombin complex concentrates infusion, two therapeutic options already used in pregnancy.
Practitioners should be aggressive in recommending effective birth control in patients with the kyphoscoliotic form of Ehlers-Danlos syndrome. In cases of established pregnancy, patients should be made fully aware of their risks of death and severe complications.
Twin-to-twin transfusion syndrome (TTTS) has been related to unbalanced unidirectional arteriovenous anastomoses in the placenta of monochorionic diamniotic (DiMo) twin gestations. As maternal malnutrition accounting for hypoproteinemia and anemia has been detected in severe cases of TTTS, the purpose of this study was to evaluate the impact of early diet supplementation on TTTS. Fifty-one DiMo twin pregnancies were given commercially available oral nutritional diet supplements and then compared in a retrospective cohort study to 52 twin gestations with the same chorionicity but not subjected to nutritional supplementation. Diet supplementation was associated with lower overall incidence of TTTS (20/52 versus 8/51, P = 0.02) and with lower prevalence of TTTS at delivery (18/52 versus 6/51, P = 0.012) when compared with no supplementation. Nutritional intervention also significantly prolonged the time between the diagnosis of TTTS and delivery (9.4 +/- 3.7 weeks versus 4.6 +/- 6.5 weeks; P = 0.014). The earlier nutritional regimen was introduced, the lesser chance of detecting TTTS ( P = 0.001). Although not statistically significant, dietary intervention was also associated with lower Quintero stage, fewer invasive treatments, and lower twin birth weight discordance. Diet supplementation appears to counter maternal metabolic abnormalities in DiMo twin pregnancies and improve perinatal outcomes in TTTS when combined with the standard therapeutic options.
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