Asunto(s)
Neoplasias , Anomalías Cutáneas , Anciano , Femenino , Antebrazo , Humanos , Piel , Colgajos Quirúrgicos , Extremidad SuperiorRESUMEN
Schöpf-Schulz-Passarge syndrome (SSPS) is a rare ectodermal dysplasia characterized by cysts of the eyelids, hypodontia, hypotrichosis, palmoplantar keratosis and onychodystrophy, and it is not common in Asia according to the published work. This autosomal recessive disorder was believed to result from mutations in the WNT10A gene. We report a 54-year-old Taiwanese man with SSPS resulted from a homozygous mutation (p.Arg104Cys) in WNT10A. This mutation has not been reported in odonto-onycho-dermal dysplasia but was demonstrated to link with dental abnormalities. This report implies the significance of WNT10A gene mutation in ectodermal dysplasia and highlights the clinical features of SSPS.
Asunto(s)
Anodoncia/genética , Glándulas Ecrinas/anomalías , Neoplasias de los Párpados/genética , Hipotricosis/genética , Queratodermia Palmoplantar/genética , Proteínas Wnt/genética , Anodoncia/diagnóstico , Anodoncia/patología , Biopsia , Glándulas Ecrinas/patología , Neoplasias de los Párpados/diagnóstico , Neoplasias de los Párpados/patología , Homocigoto , Humanos , Hipotricosis/diagnóstico , Hipotricosis/patología , Queratodermia Palmoplantar/diagnóstico , Queratodermia Palmoplantar/patología , Masculino , Persona de Mediana Edad , Mutación Missense , Linaje , Piel/patología , TaiwánRESUMEN
Ordered mesoporous TiO(2) materials with an anatase frameworks have been synthesized by using a cationic surfactant cetyltrimethylammonium bromide (C(16)TMABr) as a structure-directing agent and soluble peroxytitanates as Ti precursor through a self-assembly between the positive charged surfactant S(+) and the negatively charged inorganic framework I(-) (S(+)I(-) type). The low-angle X-ray diffraction (XRD) pattern of the as-prepared mesoporous TiO(2) materials indicates a hexagonal mesostructure. XRD and transmission electron microscopy results and nitrogen adsorption-desorption isotherms measurements indicate that the calcined mesoporous TiO(2) possesses an anatase crystalline framework having a maximum pore size of 6.9 nm and a maximum Brunauer-Emmett-Teller specific surface area of 284 m(2) g(-1). This ordered mesoporous anatase TiO(2) also demonstrates a high photocatalytic activity for degradation of methylene blue under ultraviolet irradiation.
RESUMEN
Nanocrystalline TiO(2) rods and hollow tubes with an engraved pattern on the surface have been prepared by a novel anionic template-assisted sol-gel synthesis via urea treatment and under hydrothermal condition. X-ray diffractometry (XRD) results indicate that these nanocrystallines consist predominantly of anatase TiO(2), with minor amounts of rutile and brookite. Scanning and transmission electron microscopy (SEM and TEM) analyses reveal these rods and hollow tubes may result from the aggregates of nanorods of approximately 10 nm in diameter. The crystallographic faceting found from TEM further reveals the polymorphic nature of the nanocrystalline TiO(2) thus prepared. A "reverse micelle" formation mechanism taking into account the hydrothermal temperature, the pH effect of the sol-gel system, the isoelectric point, the formation of micelles, and the electrostatic interaction between the anionic surfactant and the growing TiO(2) particulates is proposed to illustrate the competition between the physical micelle assembly of the ionic surfactants and the chemical hydrolysis and condensation reactions of the Ti precursors.
