Primigravida with XXXX : a case report.

@entity1 @entity3742 @entity548 @entity3459 @entity74
BACKGROUND: @entity3742 is a rare @entity3459 , mainly inherited in an autosomal recessive pattern. It is characterized by a @entity74 on one of the four genes encoding the subunits of the transmembrane protein complex GPIb-V-IX, physiologically expressed only in platelets. The exact phenotype varies widely from individual to individual depending on the particular mutation presented. Currently, there is no consensus about ideal management of affected pregnant @entity1 , in face of the scarcity of cases. CASE PRESENTATION: We report on a 28-year-old Black Brazilian primigravida who was referred to our maternity hospital, a tertiary care center, for decision about the most adequate mode of delivery. She was admitted with a platelet count of 43.000 plt/ L, and hemoglobin of 13.6  g/dL. Platelet transfusion was regarded as a necessary prophylactic measure prior to delivery. Ten units of random donor platelets were administered on the course of three days, after which the @entity1 was submitted to an elective cesarean section delivery under general anesthesia at 40  weeks of gestational age. A healthy male baby with a normal birthweight of 3.615  kg was delivered. After the delivery, the mother's state continued being assessed daily, with special attention taken to lochia and surgical wound healing. At one week postpartum, a complete blood count revealed a platelet count of 41.000 plt/ L, and hemoglobin of 13.3  g/dL. As there were no signs of neither evident nor @entity548 , and surgical wound was healing accordingly, the @entity1 was discharged, after being oriented about @entity548 preventive measures. CONCLUSION: The peripartum period is regarded as the most crucial moment of pregnancy in @entity1 with @entity3742 , hence the importance of a judiciously planned mode of delivery, and of careful prophylaxis against @entity548 beforehand. Furthermore, absence of complications during the peripartum period does not predict how the @entity1 will do subsequently. Strict vigilance is warranted at least until six weeks postpartum, due to the virtual risk of secondary postpartum @entity548 .
