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.

Primer consenso venezolanode enfermedad por reflujo gastroesofágico / Venezuelan consensus on gastroesophageal reflux diseases

El Consenso Venezolano de Enfermedad por Reflujo Gastroesófágico (ERGE) se realizó con el objeto de proveer guías para mejorar la identificación, el diagnóstico y el tratamiento de este trastorno en el país. Los coordinadores establecieron las líneas de consenso, basado en una revisión sistemática de la literatura médica de los últimos 15 años a partir de 1995. Participaron 55 miembros con el aval de sus respectivas cátedras y sociedades locales de gastroenterología. Éstos revisaron y presentaron los temas con sus niveles de evidencia y grados de recomendación para discutirlos y votarlos en una reunión plenaria. Tras un informe final de los miembros, los coordinadores prepararon las declaraciones finales. El consenso concluyó que la enfermedad por reflujo gastroesofágico tiene una prevalencia estimada del 11,54% en Venezuela, a predominio del sexo femenino (Grado B). El diagnóstico es fundamentalmente clínico, basado en la presencia de síntomas típicos y/o atípicos, e incluso síntomas de alarma que sugieren alguna complicación (Grado B). La endoscopia es importante en la investigación de la presencia de esofagitis de reflujo y laringitis de reflujo (Grado B). Las otras pruebas diagnósticas como lo son la pHmetría esofágica de 24 horas con o sin impedancia intraluminal multicanal son importantes en los pacientes que no tienen esofagitis, tienen síntomas atípicos o cuando hay falla en la respuesta al tratamiento médico (Grado B). La radiología, manometría esofágica y el ultrasonido endoscópico no están indicados en el diagnóstico de la ERGE (Grado B). El objetivo del tratamiento es reducir la exposición ácida en el esófago y con esto: aliviar los síntomas, cicatrizar las lesiones en la mucosa esofágica, prevenir la recurrencia y las complicaciones. Los inhibidores de bomba de protones deberían ser la primera opción en el tratamiento en el síndrome de ERGE y en la esofagitis por reflujo tanto en la fase aguda como durante el mantenimiento...

The Venezuelan Gastroesophageal Reflux Disease (GERD) Consensus was carried out in order to provide guidelines to improve the identification, diagnosis and treatment of this illness in Venezuela.  The coordinators established the consensuslines, based on a systematic revision of the medical literature of the last 15 years starting from 1995. 55 physicians participated with the support of their respective medical schools and local societies. They revised and presented the topics with their respective evidence levels and recommendation grades to discuss and vote them in a plenary meeting. After a final report of the members, the coordinators prepared the definitive declarations. The consensus concluded that GERD prevalence in Venezuela is 11,54%, higher in women than men (Grade B). The diagnosis is mainly clinical, based on the presence of typical and/or atypical symptoms and alarm symptoms that may suggest the presence of complications (Grade B). Endoscopy is important when reflux esophagitis and laryngitis are present (Grade B). Other diagnostic tests as ambulatory 24 hours pH monitoring with or without multichannel intraluminal impedance are important in patients without esophagitis, with atypical symptoms or when there is flaw in the answer to the medical treatment (Grade B). Radiology, esophageal manometry and endoscopic ultrasonography are not indicated in the diagnosis of GERD (Grade B). The objective of the treatment is to reduce the presence of acid in the esophagus and consequently: alleviate the symptoms and heal lesions in the esophagus mucosa to prevent recurrence and complications. Proton pump inhibitors should be the first option drug in the treatment of GERD syndrome andin esophagitis during the acute and the maintenance phase using standard or half dose (Grade A). So far, pokinetics are drugs with a limited use in GERD patients; they stimulate the esophagus/gastric motility...

Antidiabetic activities of Tecoma stans (L.) Juss. ex Kunth.

ETHNOPHARMACOLOGICAL RELEVANCE: Tecoma stans aqueous extract (TAE) is widely used as a traditional antidiabetic remedy in Mexico; its rational use is controversial. We provide evidence of its main antidiabetic activities. AIM OF THE STUDY: To evaluate in vivo and in vitro intestinal alpha-glucosidases inhibition as the possible mode of action of TAE on type 2 diabetes mellitus (DM2) animal models, and to test the effects of its sub-chronic administration on lipids and glucose blood levels. MATERIALS AND METHODS: In healthy and streptozotocin (STZ)-induced diabetic male Sprague-Dawley rats, glucose or cornstarch was administered after an oral dose of TAE, acarbose, tolbutamide or vehicle, in order to build starch and glucose tolerance curves (STC and GTC). An intestinal brush border preparation was used to evaluate the TAE alpha-glucosidases inhibitory activity. Moreover, in STZ-induced diabetic rats TAE, tolbutamide or vehicle was administered for 21 days for evaluate their effects on fasting glucose cholesterol and triglycerides. Also, TAE total phenolic compounds were quantified. RESULTS: In STC, TAE decreased hyperglycemic peak values in both healthy and STZ-treated rats, in a magnitude similar to that of acarbose. The in vitro preparation showed a dose-dependent inhibition of glucose release from starch. Sub-chronic administration of TAE significantly reduced cholesterol and triglycerides levels. Moreover, we confirmed that acute and sub-chronic administration of TAE (500mg/kg) in both rat models did not diminish fasting glucose and did not modify the GTC. CONCLUSIONS: The study present evidence that the main antidiabetic effect of TAE is due to intestinal alpha-glucosidase inhibition by decreasing the postprandial hyper-glycaemia peak; in addition, TAE sub-chronic administration reduces triglycerides and cholesterol, without modifying fasting glucose.

[Onychomycosis due to Malassezia.]. / Onicomicosis por Malassezia.

The records of fifty patients presenting Malassezia spp. associated onychomycosis were compiled from two different mycology laboratories from Medellín, Colombia. Malassezia spp. was isolated by culture as the only etiological agent in 32% of the cases and associated to a yeast of the genus Candida in 30% of the cases. In 22% of the cases although Malassezia spp. was observed by direct examination, it was no isolated but others species were obtained. No etiological agent was isolated by culture in 16% of the cases. We found evidence of the Malassezia spp.- Candida relationship in 48% of the cases by either direct examination or by culture isolation. The level of detection of Malassezia spp. by culture isolation was of 62% as compared to the direct examination. Results showed similar patterns of distribution of epidemilogical factors for both entities: onychomycosis by Candida albicans and onychomycosis by Malassezia spp.

Evaluation of the calcium-antagonist, antidiarrhoeic and central nervous system activities of Baccharis serraefolia.

Baccharis serraefolia is a widely used plant to treat diarrhoea in Mexican traditional medicine. Although the methanolic extract of this plant has shown an important dose-dependent spasmolytic activity, its underlying mechanism has not been studied. In the present work, the methanolic extract of B. serraefolia significantly delayed the onset of tonic seizures induced by strychnine and pentylenetetrazol; besides, it diminished the death rate and number of animals that exhibited convulsions. It produced potentiation of the hypnotic effect of pentobarbital. Oral administration produced an inhibition of gastrointestinal transit in mice as effective as that produced by loperamide. As to the effect on smooth muscles, the active extract produced an inhibition of contraction induced electrically, which could not be reversed by naloxone. The calcium concentration-contraction curve showed a rightward displacement when the extract was added to isolated guinea pig ileum depolarized with high K+ and cumulative concentrations of Ca2+. The results suggest that the methanolic extract does not interact with classical opiate receptors and its effects, at least that produced on smooth muscle, may be due to a probable interference with calcium influx and/or calcium release from an intra-cellular store.

Papiloma nasal invertido en enfermo de lepra

Se presenta un caso de Papiloma invertido malignizado de mucosa nasal con invasión de senos paranasales y órbita, recidivante a la terapéutica quirúrgica y radioterapia.

Resistencia sulfonica de tres casos de lepra lepromatosa

Se presentan tres nuevos casos de lepromatosos avanzados, inactivados durante muchos años y con recidiva clínico-bacteriológico como consecuencia de irregularidad y dosis insuficientes en el tratamiento. Se hacen consideraciones sobre la forma de evitar la sulfonoresistencia, cada día más frecuente, empleando dosis iniciales altas e asociando clofazimina.