RESUMEN
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. Laboratory testing confirmed the kyphoscoliotic type. Based on clinical and phenotypic similarities with the vascular type of Ehlers-Danlos syndrome, termination was advised. Minor trauma in the third trimester led to delivery of a stillborn fetus, which was followed by disseminated intravascular coagulopathy and death of the mother. Maternal autopsy revealed that there had been a spontaneous rupture of the right iliac artery. CONCLUSION: 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.
Asunto(s)
Síndrome de Ehlers-Danlos/complicaciones , Complicaciones del Embarazo , Adolescente , Aneurisma Roto , Cesárea , Coagulación Intravascular Diseminada , Síndrome de Ehlers-Danlos/fisiopatología , Resultado Fatal , Femenino , Humanos , Arteria Ilíaca/patología , Cifosis/etiología , Cooperación del Paciente , Embarazo , Complicaciones del Embarazo/fisiopatología , Rotura Espontánea , Escoliosis/etiología , MortinatoRESUMEN
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. Her persistent symptomatology ultimately led to a video-assisted thoracoscopy and thorascopic decortication. The diagnosis was established by pathology and immunohistochemistry of the cells, which were consistent with primary pulmonary synovial sarcoma. CONCLUSION: Malignancies, even those as uncommon as primary synovial sarcoma, should be considered in the differential diagnosis of pneumothorax during pregnancy.
Asunto(s)
Neoplasias Pulmonares/diagnóstico , Neumotórax/etiología , Complicaciones Neoplásicas del Embarazo/diagnóstico , Sarcoma Sinovial/diagnóstico , Adulto , Femenino , Humanos , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/terapia , Neumotórax/diagnóstico , Neumotórax/terapia , Embarazo , Complicaciones Neoplásicas del Embarazo/etiología , Complicaciones Neoplásicas del Embarazo/terapia , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/terapiaRESUMEN
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.
Asunto(s)
Sacarosa en la Dieta/uso terapéutico , Suplementos Dietéticos , Transfusión Feto-Fetal/prevención & control , Embarazo Múltiple , Gemelos Monocigóticos , Adulto , Proteínas Sanguíneas/análisis , Estudios de Cohortes , Femenino , Transfusión Feto-Fetal/epidemiología , Alimentos Formulados , Hematócrito , Hemoglobinas/análisis , Humanos , Incidencia , Estado Nutricional , Embarazo , Resultado del Embarazo , Atención Prenatal , Estudios Retrospectivos , Albúmina Sérica/análisis , Ultrasonografía PrenatalRESUMEN
The majority of bariatric surgical procedures are performed in young women. There is a concern about safety and outcomes of pregnancies after weight loss surgery. Pregnancy after weight loss surgery is not only safe, but is associated with more favorable outcomes in comparison to obese populations who do not undergo weight loss surgery. An interval of 2 years is recommended from surgery to pregnancy. This delay helps avoid most of the potential nutritional complications. Optimal patient care is achieved in an experienced, multidisciplinary center. Early involvement of the bariatric surgeon in evaluating abdominal pain is critical because the underlying pathology may relate to the previous weight loss surgery. Although infertility is improved after weight loss surgery, reliable modes of contraception may be limited in this population.
Asunto(s)
Cirugía Bariátrica/efectos adversos , Infertilidad , Obesidad Mórbida/cirugía , Complicaciones Posoperatorias , Complicaciones del Embarazo , Resultado del Embarazo , Dolor Abdominal/etiología , Femenino , Humanos , Estado Nutricional , Obesidad Mórbida/complicaciones , Síndrome del Ovario Poliquístico , EmbarazoRESUMEN
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.