We reported on 16 new Brazilian patients and review findings in 12 previously reported cases (25 apparently unrelated Brazilian families) from Hospital of Rehabilitation of Craniofacial Anomalies, presenting with Richieri-Costa-Pereira syndrome. All patients display a unique pattern of anomalies consisting of microstomia, micrognathia, abnormal fusion of mandible, cleft palate/Robin sequence, absence of central lower incisors, minor ears anomalies, hypoplastic first ray, abnormal tibiae, hypoplastic halluces, and clubfeet. Learning disability was also a common finding. The sex-ratio showed deviation toward to female (1.8F:1M). Recurrence in sibs was observed in nine instances and consanguinity in 11, supporting the hypothesis of autosomal recessive inheritance. Nineteen of the 25 families lived in São Paulo State, seven of them (10 affected individuals) from an isolated region named ''Vale do Ribeira.'' The geographic barrier of this region associated with the high incidence of the consanguineous matting suggested that this condition is caused by a rare mutation with a founder effect. With the exception of one patient in France, all known cases are of Brazilian origin. The causative gene of this rare syndrome remains unknown. Ó

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Background On March 2020, the World Health Organization declared the coronavirus disease 2019 outbreak a pandemic. During this period, surgical activity and admission to the Emergency Department (ED) decreased globally. The aim of this article is to understand how the admission of a patient to the ED for vascular surgery changed in our center in Portugal and if this situation prevented urgent surgical procedures. Methods Through a retrospective study, we compared the volume of patients admitted to the ED during the emergency state (ES) in Portugal with the same period in 2019. In addition, we analyzed the urgent surgical activity during the ES and in the correspondent period of the previous 10 years, regarding limb acute ischemia, acute aortic pathology, and vascular trauma. Two groups of patients were formed—patients operated during the ES and during the non-ES, for control. Statistical analysis was performed using IBM SPSS® Statistics, version 25. Results In the ES, 115 patients were observed at the ED and 179 in the 2019 corresponding period. During the ES, patients significantly recurred less to the ED directly from home ( P < 0.001) and were less referred to the ED by primary care doctors ( P < 0.001). Patients observed at the ED were significantly more urgent—required urgent surgery or were admitted to the department—than those in 2019 (40% vs. 24%). However, there were no differences when only considering urgent surgery (14% in ES vs. 10% in 2019). In the ES, 38% of patients observed at the ED were discharged with no follow-up related to vascular surgery against 60% in 2019, although this difference was not significant. Compared with the preceding 10 years, there are not significant differences in the number of patients who underwent urgent surgery in both ES and non-ES periods. In patients with acute limb ischemia, we did not find an increase in the time between onset of symptoms and ED admission, during the ES. Conclusions Fewer patients were admitted at the ED during the ES, and those admitted were significantly more urgent. We did not find a decrease in the number of urgent surgeries when compared with the preceding 10 years. Therefore, we cannot assume that coronavirus pandemic precluded urgent surgical procedures.

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