A novel Pyrin-Associated Autoinflammation with Neutrophilic Dermatosis mutation further defines 14-3-3 binding of pyrin and distinction to Familial Mediterranean Fever.

OBJECTIVE: Pyrin-Associated Autoinflammation with Neutrophilic Dermatosis (PAAND) is a recently described monogenic autoinflammatory disease. The causal p.S242R MEFV mutation disrupts a binding motif of the regulatory 14-3-3 proteins within pyrin. Here, we investigate a family with clinical features consistent with PAAND in whom the novel p.E244K MEFV mutation, located in the +2 site of the 14-3-3 binding motif in pyrin, has been found. METHODS: Multiplex cytokine analyses were performed on p.E244K patient and control serum. Peripheral blood mononuclear cells were stimulated ex vivo with lipopolysaccharide (LPS). In vitro, inflammasome complex formation was evaluated by flow cytometry of Apoptosis-associated Speck-like protein containing a Caspase recruitment domain (ASC) specks. Interleukin-1ß (IL-1ß) and IL-18 production was quantified by ELISA. The ability of the p.E244K pyrin mutation to interact with 14-3-3 was assessed by immunoprecipitation. RESULTS: PAAND p.E244K patient serum displayed a different cytokine profile compared with patients with Familial Mediterranean Fever (FMF). In overexpression models, p.E244K pyrin was associated with decreased 14-3-3 binding and increased ASC speck formation. THP-1 monocytes expressing PAAND pyrin mutations demonstrated spontaneous caspase-1-dependent IL-1ß and IL-18 secretion, as well as cell death, which were significantly greater than those of wild-type and the FMF-associated mutation p.M694V. CONCLUSION: In PAAND, disruption of the +2 position of a 14-3-3 binding motif in pyrin results in its constitutive activation, with spontaneous production of IL-1ß and IL-18, associated with inflammatory cell death. The altered serum cytokine profile may explain the different clinical features exhibited by PAAND patients compared with those with FMF.

Results of the implementation of a double-check protocol with point-of-care ultrasound for acute heart failure in the emergency department.

OBJECTIVE: To determine the effectiveness of a double-check protocol using Point-of-Care Ultrasound in the management of patients diagnosed with Acute Heart Failure in an Emergency Department. METHOD: Prospective analytical cross-sectional observational study with patients diagnosed with Acute Heart Failure by the outgoing medical team, who undergo multi-organ ultrasound evaluation including cardiac, pulmonary, and inferior vena cava ultrasound. RESULTS: 96 patients were included. An alternative diagnosis was found in 33% of them. Among the 77% where AHF diagnosis was confirmed, 73.4% had an underlying cause or condition not previously known (Left Ventricular Ejection Fraction less than 40% or moderate-severe valvulopathy). The introduction of the protocol had a clinically relevant impact on 47% of all included patients. CONCLUSIONS: The implementation of a double-check protocol using POCUS, including cardiac, pulmonary, and inferior vena cava assessment in patients diagnosed with Acute Heart Failure, demonstrates a high utility in ensuring accurate diagnosis and proper classification of these patients.

Lipoprotein lipase expression in livers of morbidly obese patients could be responsible for liver steatosis.

BACKGROUND: Most patients with morbid obesity develop non-alcoholic fatty liver disease (NAFLD). The origins of lipid deposition in the liver and the effects of bariatric surgery in the obese with NAFLD are controversial. METHODS: We analyzed lipids and lipoprotein lipase (LPL) in both plasma and liver biopsies performed before and 12-18 months after Roux-en-Y gastric bypass surgery in 26 patients. RESULTS: In the livers of morbidly obese patients, the levels of LPL messenger RNA (mRNA) were higher (4.5-fold) before surgery than afterwards than control livers. In these patients, LPL activity was also significantly higher (91 +/- 7 mU/g) than in controls (51 +/- 3 mU/g, p = 0.0026) and correlated with the severity of the liver damage. All hepatic lipids were significantly increased in obese patients; however, after bariatric surgery, these lipids, with the exception of NEFA, tended to recover to normal levels. CONCLUSIONS: The liver of obese patients presented higher LPL activity than controls, and unlike the controls, this enzyme could be synthesized in the liver because it also present LPL mRNA. The presence of the LPL activity could enable the liver to capture circulating triacylglycerides, thus favoring the typical steatosis observed in these patients.

Influence of apical constriction diameter on Root ZX apex locator precision.

Small-diameter hand files are systematically used to determine the initial working length and to establish patency of the root canal. In wide canals, electronic apex locator accuracy might be influenced by file size. This article reports on a study to assess the influence of apical constriction diameter on the precision of the Root ZX apex locator by using files of varying diameter on teeth with 3 different degrees of apical widening: 0.37, 0.62, and 1.02 mm. Ten extracted single-root teeth, stored in saline solution at 37 degrees C, were sectioned at the enamel-cement junction and embedded in an alginate mold. Initial working length (L0) was determined with a #10 Kerr file, and canals were irrigated with either 50% citric acid or 5.25% sodium hypochlorite. To assess the ability of the locator to identify the narrowest area of the root canal as constriction diameter increased, the canal was progressively widened by insertion of files of increasing diameter (10-100) at a point L0+1 mm. After each enlargement, the working length (L) was redetermined, with files from #10 (L10) up to the file used for widening. Comparison of statistical results was based on the difference between final working length and initial length (L10) for each apical width. Statistical analysis was carried out with analysis of variance test for comparison of means. Results showed that at apical constriction widths of 0.37 and 0.62 mm, there was no significant difference between initial working lengths as determined by a Kerr #10 file and final working lengths after widening with files of up to #60. In those teeth whose apical width had been increased to 1.02 mm, there was no statistically significant difference between initial and final working lengths as measured by files from #10-#25; however, significant differences were apparent between #10 and #30, #35, or #40 (P<.05), and the degree of significance increased considerably (P<.001) for files #45 or greater. These results suggest that Root ZX apex locator precision varies as a function of apical constriction diameter.

Consensus document on the use of antibiotic prophylaxis in dental surgery and procedures.

The goal of antibiotic prophylaxis in Odontology is to prevent the onset of infections through the entranceway provided by the therapeutic action, therefore it is indicated providing there is a considerable risk of infection, either because of the characteristics of the operation itself or the patient s local or general condition. Nonetheless, clinical trials with antibiotics in dental pathologies have had scant regard for the required methodological criteria and, in addition, are not sufficiently numerous. This text presents the results of an expert conference comprising the Presidents of the most representative Scientific Societies in Spain who have analyzed the existing literature and have drawn on their valuable professional experience. It describes the technical circumstances, analyzes the biological and pharmacological foundations and their application to the most representative medical situations. It is concluded that antibiotic prophylaxis in Odontology has certain well-founded, precise indications and offers the international scientific community a practical protocol for action.

Síndrome de la cimitarra en resonancia magnética cardiovascular / Scimitar syndrome in a cardiovascular magnetic resonance scan

Resumen El síndrome de la cimitarra es una anomalía parcial del retorno venoso pulmonar de características complejas. Está asociado a múltiples malformaciones cardiovasculares y pulmonares, y representa un diagnóstico diferencial importante en los pacientes con dilatación inexplicada de las cavidades derechas. Se expone el caso de una mujer en la cuarta década de la vida, con un cuadro clínico de dolor torácico, episodios sincopales y deterioro de su clase funcional, que por sus hallazgos en la ecocardiografía fue llevada a resonancia magnética cardíaca que mostró anomalía parcial del retorno venoso pulmonar infracardiaca, hallazgo no visualizado en la ecocardiografía, y confirmó el diagnóstico específico de síndrome de la cimitarra.

Abstract Scimitar syndrome is a partial anomaly, of complex characteristics, of pulmonary venous return. It is associated with multiple cardiovascular and lung malformations, and requires a significant differential diagnosis in patients with an unexplained dilation of the right cavities. The case is presented of a woman, in the fourth decade of life, with a clinical picture of chest pain, syncopal episodes, and a deterioration of her functional class. Due to her findings in the echocardiograph, a cardiac magnetic resonance scan was performed that showed an infra-cardiac partial anomalous pulmonary venous return, a finding that was not observed in the echocardiograph, and confirmed the specific diagnosis of scimitar syndrome.

Papilomatosis biliar: presentación de un caso con trasplante hepático / Biliary Papillomatosis: A Case Report

Las neoplasias papilares intraductales de los ductos biliares (NPIB), tradicionalmente llamadas "papilomatosis biliar", son raras y se caracterizan por una apariencia macroscópica papilar y, en muchos casos, secreción visible de mucina. Ocurren con mayor frecuencia en pacientes con antecedente de litiasis biliar. Presentamos el caso de una paciente de 47 años de edad, a quien se le realizó un trasplante hepático por cirrosis biliar secundaria a litiasis intra y extrahepática, quien presentó durante muchos años episodios de colangitis recurrente, los cuales fueron manejados entre otros, con una anastomosis bilioentérica. En los estudios de imagen previos al trasplante, además de los cambios por cirrosis, se encontró una importante dilatación de la vía biliar intra y extrahepática, con cálculos de la vía biliar. La patología del explante mostró papilomatosis de la vía biliar. La paciente desarrolló recurrencia temprana en la vía biliar extrahepática nativa. Ha recibido manejo conservador hasta este momento.

Intraductal papillary neoplasias of the bile ducts (IPNB), traditionally called "biliary papillomatosis", is a rare entity characterized by macroscopic papillary appearance and in many cases visible secretion of mucin. It occurs more frequently in patients with history of biliary lithiasis. We present the case of a 47 years old patient who underwent liver transplantation due to biliary cirrhosis secondary to intra- and extrahepatic lithiasis, which suffered for many years episodes of recurrent cholangitis managed among others, with a bilioenteric anastomosis. At pre-transplant imaging studies, in addition to changes due to cirrhosis, a significant intra- and extra- hepatic biliary tract dilation was found with multiple calculi within. Pathology results reported the presence of bile duct papillomatosis. The patient developed early recurrence into the native extrahepatic bile duct. She has so far received conservative management.

Critical diameter of apical foramen and of file size using the Root ZX apex locator: an in vitro study.

INTRODUCTION: An evaluation was made of the accuracy of the Root ZX apex locator (J. Morita Corp, Tokyo, Japan) in widened foramina, considering the existing controversy over this issue in the literature. METHODS: Ten single-root teeth were embedded in an alginate mold. The foramina were widened from 0.6 mm to 1.0 mm. The measurements were taken with all possible file sizes ≥#10. The statistical accuracy of the Root ZX was calculated for the different diameters and for the influence of file size. RESULTS: The accuracy of the Root ZX apex locator with a range of error of ±0.5 mm was 87% in an apical foramen size of 0.6 mm and 84% using files size 45 or larger in an apical foramen size of 0.7 mm. With a tolerance of ±1 mm, the accuracy was 99% in an apical foramen size of 0.6 mm, 98% using files size 45 or larger in an apical foramen size of 0.7 mm, and 95% using files size 70 or larger in an apical foramen size of 0.8 mm. In the rest, accuracy was not certain. The measurements taken with smaller files were shorter. There were no cases of overestimation of the working length. CONCLUSIONS: The Root ZX apex locator was accurate for an apical size of 0.6 mm, independently of the file size; between 0.7 to 0.8 mm, we should adjust the files to the foramen, whereas above size 0.9 mm the locator is not accurate. The results show that the accuracy of this electronic apex locator is gradually lost as the foramen widens. Considering the stable conditions of in vitro studies, our findings advise caution in clinical application of the locator.

Developments in polymerization lamps.

Polymerization shrinkage of composite resins and the consequent stress generated at the composite-tooth interface continue to pose a serious clinical challenge. The development of high-intensity halogen lamps and the advent of curing units providing higher energy performance, such as laser lamps, plasma arc units, and, most recently, light-emitting diode (LED) curing units, have revolutionized polymerization lamp use and brought major changes in light-application techniques. A comprehensive review of the literature yielded the following conclusions: (1) the most reliable curing unit for any type of composite resin is the high-density halogen lamp, fitted with a programming device to enable both pulse-delay and soft-start techniques; (2) if any other type of curing unit is used, information must be available on the compatibility of the unit with the composite materials to be used; (3) polymerization lamp manufacturers need to focus on the ongoing development of LED technology; (4) further research is required to identify the most reliable light-application techniques.