A maternal and perinatal mortality in pregnancy complicated by the kyphoscoliotic form of Ehlers-Danlos syndrome.

Background: Ehlers-Danlos syndrome is a group of inherited connective tissue diseases demonstrating autosomal-dominant, autosomal-recessive, and X-linked inheritance patterns. The diagnosis can be established by clinical, biochemical, and genetic findings.

Case: Our nulliparous patient presented with an unspecified diagnosis of Ehlers-Danlos syndrome.

Nutritional supplementation in monochorionic diamniotic twin pregnancies: impact on twin-twin transfusion syndrome.

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.

Plasma exchange in a case of severe factor X deficiency in pregnancy: critical review of the literature.

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.

Pulmonary synovial sarcoma presenting as a pneumothorax during pregnancy.

Background: Synovial sarcoma is a clinically rare, but morphologically well-defined neoplasm, which accounts for approximately 10% of all malignant soft-tissue tumors. The diagnosis can be established with clinical and imaging evaluations together with immunohistochemical, electron microscopy, and molecular genetic studies.

Case: We describe a case of primary pulmonary synovial sarcoma presenting as a pneumothorax in a young woman at 34 weeks of gestation.