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Monogenic autoinflammatory diseases (AIFD) are rare disorders characterized by an uncontrolled increase of the systemic inflammatory response, which is caused by mutations in genes involved in inflammatory pathways. Over the last few years, new genes and proteins responsible for new monogenic AIFD have been identified and a substantial improvement in their treatment has been achieved. Monogenic AIFD manifestations typically begin during childhood, but they can also occur in adults. Compared to pediatric patients, adults usually present with a less severe disease and fewer long-term complications. In addition, patients with adult-onset disease carry low-penetrance mutations more often than pathogenic variants. A late-onset of AIFD may be occasionally associated with the presence of somatic mutations. In this study, we review the most frequent monogenic AIFD, and others recently described, which may occur during adulthood., (Copyright © 2017 Elsevier España, S.L.U. All rights reserved.)

Eighty three primigravidae patients at the end of latency labor, erased cervix, 3 cm dilation, vertex presentation and adequate pelvis, were studied. Two groups were formed: 53 patients in the study group, who received active management of labor, and 30 patients in the control group, treated in the traditional way. In all the patients a graphic recording of labor, was carried out; it included all the events, and as labor advanced, a signoidal curve of cervical dilatation, was registered, as well as the hyperbolic one for presentation descent. The study group received the method in a systematized manner, as follows: 1. Peridular block. 2. Amniotomy. 3. IV oxytocin one hour after amniotomy. 4. FCR monitoring. 5. Detection of dystocia origin. Materno-fetal morbidity was registered in both groups, as well as cesarean section rate, instrumental delivery and its indications, labor duration, and time of stay in labor room. Diminution of above intems and opportune detection of dystocia, were determined. It was concluded that a constructive action plan, starting at hospital admission in most healthy women, allows a normal delivery of brief duration.