RESUMEN
The Marfan's syndrome is an autosomal dominant genetic disorder resulting in a diminished strength of connective tissue. The ocular, skeletal and cardiovascular systems are mostly at risk. Principal complications of the disease are aortic dilatation and the risk of acute dissection. Pregnancy increases this risk. Based on the experience of our obstetrics department and after reviewing medical literature, we have tried to establish guidelines for obstetric care adapted to pregnant patients affected by Marfan's syndrome. Women with aortic root > 40 mm should avoid pregnancy. In all cases B-adrenergic receptor blockers must be used as soon as possible. Because there is a 50% risk that offspring will inherit the syndrome, prenatal diagnosis should be suggested. In addition to usual pregnancy monitoring, echocardiography should be performed every 3 months as well as 2 months after delivery. No increase of obstetrical complications has been shown in these patients. Route of delivery also depends on the diameter of the aortic root: vaginal delivery is warranted if the aortic root is<40; cesarean section should be performed in the other cases. Thus, with appropriate supervision, women with Marfan's syndrome will tolerate pregnancy without any adverse effect.
Asunto(s)
Síndrome de Marfan/complicaciones , Complicaciones del Embarazo , Antagonistas Adrenérgicos beta/uso terapéutico , Adulto , Aorta/diagnóstico por imagen , Aorta/patología , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/tratamiento farmacológico , Enfermedades de la Aorta/etiología , Parto Obstétrico/métodos , Femenino , Humanos , Síndrome de Marfan/diagnóstico por imagen , Síndrome de Marfan/terapia , Embarazo , Complicaciones del Embarazo/terapia , Resultado del Embarazo , UltrasonografíaRESUMEN
The Salvador/Warts/Hippo (Hippo) signaling pathway defines a novel signaling cascade regulating cell contact inhibition, organ size control, cell growth, proliferation, apoptosis and cancer development in mammals. The upstream regulation of this pathway has been less well defined than the core kinase cassette. KIBRA has been shown to function as an upstream member of the Hippo pathway by influencing the phosphorylation of LATS and YAP, but functional consequences of these biochemical changes have not been previously addressed. We show that in MCF10A cells, loss of KIBRA expression displays epithelial-to-mesenchymal transition (EMT) features, which are concomitant with decreased LATS and YAP phosphorylation, but not MST1/2. In addition, ectopic KIBRA expression antagonizes YAP via the serine 127 phosphorylation site and we show that KIBRA, Willin and Merlin differentially regulate genes controlled by YAP. Finally, reduced KIBRA expression in primary breast cancer specimens correlates with the recently described claudin-low subtype, an aggressive sub-group with EMT features and a poor prognosis.
Asunto(s)
Transición Epitelial-Mesenquimal , Factor de Crecimiento de Hepatocito/metabolismo , Péptidos y Proteínas de Señalización Intracelular/metabolismo , Proteínas Nucleares/metabolismo , Fosfoproteínas/metabolismo , Proteínas Serina-Treonina Quinasas/metabolismo , Proteínas Proto-Oncogénicas/metabolismo , Factores de Transcripción/metabolismo , Apoptosis , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/metabolismo , Western Blotting , Mama/citología , Mama/metabolismo , Neoplasias de la Mama/genética , Neoplasias de la Mama/metabolismo , Neoplasias de la Mama/patología , Carcinoma Ductal de Mama/genética , Carcinoma Ductal de Mama/metabolismo , Carcinoma Ductal de Mama/patología , Adhesión Celular , Proteínas de Ciclo Celular , Movimiento Celular , Proliferación Celular , Células Cultivadas , Claudina-1/genética , Claudina-1/metabolismo , Proteínas del Citoesqueleto/genética , Proteínas del Citoesqueleto/metabolismo , Femenino , Perfilación de la Expresión Génica , Factor de Crecimiento de Hepatocito/antagonistas & inhibidores , Factor de Crecimiento de Hepatocito/genética , Humanos , Técnicas para Inmunoenzimas , Péptidos y Proteínas de Señalización Intracelular/antagonistas & inhibidores , Péptidos y Proteínas de Señalización Intracelular/genética , Proteínas de la Membrana/genética , Proteínas de la Membrana/metabolismo , Neurofibromina 2/genética , Neurofibromina 2/metabolismo , Proteínas Nucleares/antagonistas & inhibidores , Proteínas Nucleares/genética , Análisis de Secuencia por Matrices de Oligonucleótidos , Fosfoproteínas/antagonistas & inhibidores , Fosfoproteínas/genética , Fosforilación , Proteínas Serina-Treonina Quinasas/antagonistas & inhibidores , Proteínas Serina-Treonina Quinasas/genética , Proteínas Proto-Oncogénicas/antagonistas & inhibidores , Proteínas Proto-Oncogénicas/genética , ARN Mensajero/genética , ARN Interferente Pequeño/genética , Reacción en Cadena en Tiempo Real de la Polimerasa , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Serina-Treonina Quinasa 3 , Transducción de Señal , Factores de Transcripción/antagonistas & inhibidoresRESUMEN
We report a case of pyometra without any related risk factor. This leads to a severe septic shock. Evolution was successful because of quick surgical management. We report literature management of such infection. Although pyometra is an uncommon disease, it should be kept in mind as delayed management may lead to fatal outcome.