Primigravida with Bernard-Soulier Syndrome: a case report.

BACKGROUND: Bernard-Soulier Syndrome is a rare congenital bleeding disorder, mainly inherited in an autosomal recessive pattern. It is characterized by a genetic defect 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 women, 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/µL, 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 patient 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/µL, and hemoglobin of 13.3 g/dL. As there were no signs of neither evident nor occult hemorrhage, and surgical wound was healing accordingly, the patient was discharged, after being oriented about bleeding preventive measures. CONCLUSION: The peripartum period is regarded as the most crucial moment of pregnancy in women with Bernard-Soulier Syndrome, hence the importance of a judiciously planned mode of delivery, and of careful prophylaxis against bleeding beforehand. Furthermore, absence of complications during the peripartum period does not predict how the woman will do subsequently. Strict vigilance is warranted at least until six weeks postpartum, due to the virtual risk of secondary postpartum hemorrhage.

Obstetric profile of perinatal deaths on a capital of the Northeast of Brazil / Perfil obstétrico dos óbitos perinatais em uma capital do Nordeste Brasileiro

Abstract Objectives: to analyze the obstetric and sociodemographic profile on perinatal deaths in Teresina the capital of Piauí, from data obtained from the Sistema de Informação de Mortalidade e Sistema de Informação de Nascidos Vivos (Brazilian Mortality Information System and Livebirth Information System). Methods: this is a retrospective cohort on perinatal deaths of mothers whose babies were born and resided in Teresina between 2010 and 2014. The analyzed variables were age and the mother´s schooling, gestational age, type of pregnancy (singleton or multiple), route of delivery (vaginal or cesarean), place of death (in and out hospital), time of death in relation to the delivery (prior, during or after), and birth weight. Results: the perinatal mortality coefficient (PMC) varied from 17.5 to 19.3 per 1,000 births. We found similarities in the sociodemographic profile and in the obstetric fetal and non-fetal deaths, both with a great incidence on 20 to 27 years-old mothers, vaginal delivery and singleton pregnancy. Low birth weight was positively related to early neonatal deaths. Conclusions: perinatal mortality presented a statistical correlation in gestational age, birth weight, and type of delivery. The PMC in our study was higher than other Brazilian capitals.

Resumo Objetivos: analisar o perfil obstétrico e sociodemográfico dos óbitos perinatais ocorridos em Teresina, capital do Piauí, a partir de dados provenientes dos Sistema de Informação de Mortalidade e Sistema de Informação de Nascidos Vivos. Métodos: coorte retrospectiva de óbitos perinatais nascidos de mães residentes em Teresina, entre 2010 e 2014. As variáveis analisadas foram faixa etária e escolaridade da mãe, idade gestacional, tipo de gravidez (única ou múltipla), via de parto (vaginal ou cesáreo), local do óbito (intra ou extra-hospitalar), momento do óbito em relação ao parto (antes, durante ou após), e peso do concepto. Resultados: o coeficiente de mortalidade perinatal (CMP) variou entre 17,5 e 19,3 por mil nascidos. Verificaram-se semelhanças quanto ao perfil sociodemográfico e obstétrico dos óbitos fetais e não fetais, ambos com maior incidência em mães com faixa etária entre 20 e 27 anos, em parto vaginal e no tipo de gravidez única. Baixo peso ao nascer se relacionou positivamente com os óbitos neonatais precoces. Conclusões: a mortalidade perinatal apresentou correlação estatística com a idade gestacional, o peso ao nascer, e o tipo de parto. O CMP no nosso estudo foi mais elevado do que o de outras capitais brasileiras.