Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years' experience of a reference centre in Spain.

BACKGROUND: Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center. MATERIALS AND METHODS: We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018. RESULTS: Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2-29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6-242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94-11.61). CONCLUSIONS: cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.

Abordaje cerrado en patología de vía aérea superior con el uso de microelectrodos: nuestra experiencia / Approach closed upper airway pathology using microelectrodes: our experience

El láser como alternativa a la cirugía abierta de la vía aérea superior ha venido a modificar la forma de abordaje de las patologías en esta área, pero no deja de ser un procedimiento costoso que no está al alcance de todos los servicios. Por este motivo se han reinventado nuevas formas de abordaje que cumplan los mismos requisitos tanto de la cirugía abierta como con láser pero con un menor coste. Presentamos una serie de 30 casos realizados en un período de 6 años por motivos tanto tumorales como no, en los que se realizaron abordajes cerrados a través de microcirugía con disección mediante microelectrodos. Obteniendo pocas complicaciones y una disminución de la estancia hospitalaria significativa. Con lo cual nos parece una técnica eficiente para abordajes de este tipo.

The laser as an alternative to open surgery of the upper airway has come to change the form of approaching the disease in this area, but it is still an expensive procedure that is not available to all services. For this reason a new ways of approach to meet the same requirements both open as laser but at a lower cost surgery. We present a series of 30 cases performed over a period of 6 years for reasons as much tumor, which closed approaches through microsurgical dissection were performed using microelectrodes. Obtaining few complications and significant decreased hospital stay. Our considerations is it seems an efficient technique for such approaches.

An SYBR Green-based real-time RT-PCR assay for the detection of H5 hemagglutinin subtype avian influenza virus.

Increasing diversity among H5 hemagglutinin (HA) subtype avian influenza (AI) viruses has resulted in the need of novel sensitive and specific molecular assays. In this study, an SYBR Green-based real-time reverse transcription-PCR (RRT-PCR) assay was developed for the detection of H5 subtype AI virus. Sequence analysis of the Mexican lineage H5N2 isolates (subgroup B) revealed several mismatches in the primer/hydrolysis probe set reported in the commonly used RRT-PCR assay for the detection of H5 North American lineage. The present assay was designed to circumvent the challenge that these viruses represent for the specific detection of H5 subtype AI viruses. This RRT-PCR assay successfully detected a range of different H5 subtype AI strains from both Eurasian and North American lineages representing different avian H5 HA clades from diverse geographical locations. The sensitivity of the present method was determined by using in vitro-transcribed RNA and 10-fold serial dilutions of titrated AI viruses. High sensitivity levels were obtained, with limits of detection of 10(0) 50% egg infectious dose (EID50)/mL and 4.2 gene copies/µl. The linear ranges of the assay span within 10(6)-10(0) EID50/mL and 10(6)-10(0) gene copies/µl. The results obtained from this method were directly compared with those of the H5 RRT-PCR assay recommended by the OIE. The comparison was performed with 110 tracheal and cloacal swabs from various bird species collected during field and laboratory investigations in Eurasia and Africa in 2006 and 2008 and showed 100% agreement. This assay is recommended as an alternative method, also allowing a 'double check' approach detection, to be use mainly in outbreak scenarios with higher risk of poultry infections by Central American/Caribbean H5 AI viruses.