Comparative Passivation of Si(100) by H2 and D2 Atmospheres under Simultaneous Xe+ Bombardment: An X-ray Photoelectron Spectroscopy Analysis.

This study presents a comparison of H2 and D2 passivation on Si(100) under simultaneous Xe+ ion bombardment. The impact of Xe+ ions causes significant damage to the substrate surface, leading to an increase in H2 (D2) retention as Si-H (Si-D) bonds. The ion bombardment conditions are precisely controlled using a Kaufman ion gun. The atomic concentrations on the surface of the sample were investigated by quasi-in situ X-ray photoelectron spectroscopy. A simple methodology is employed to estimate the H (D) chemical concentration and the cover ratio of the sample, with regard to the oxygen concentration through residual water chemisorption present in the vacuum vessel. Differences in passivation are expected when using H2 or D2 atmospheres because their retained scission energies and physisorption properties differ. The results indicate an increase of the sticking coefficient for D2 and H2 under the ion bombardment. It is also found that the flux of H2 (D2) impinging on the surface contributes to play an important role in the whole process. Finally, a model is proposed to describe the phenomenon of the passivation of Si under Xe+ ion bombardment in the presence of H2 (D2).

[Analysis of the comorbidities and role of laparoscopy in recurrent abdominal pain]. / Análisis de las comorbilidades y papel de la laparoscopia en el dolor abdominal recurrente.

INTRODUCTION AND OBJECTIVES: Recurrent abdominal pain is defined as > 3 episodes of abdominal pain accompanied by affectation of the daily activity, during > 3 months. Our objective is to analyze the role of diagnostic and/or therapeutic laparoscopy. MATERIAL AND METHODS: A descriptive, retrospective study from 2004 to 2016. Patients: <14 years with DAR who underwent laparoscopy. Variables: age, sex, history, surgical findings, histology and follow-up. RESULTS: 55 patients. Mean age: 10.7 years. Female 63, 6%. Probability of allergic comorbidity: 27.27% [16.138-40.962] (CI 95%). Probability of subsequent psychological comorbidity: 12.72% [5.27 -24.48] (95% CI). Histological changes 31/55 (56.36%): lymphoid nodular hyperplasia 10/31, appendicular inflammation 7/31, fecalite 3/31, carcinoid tumor 1/31, appendicular fibrosis 3/31, Meckel diverticulum 1/31, association of several of the above 8/31. Macroscopic alterations 31/55 (56.36%): appendicular pathology 10/31, adhesions 5/31, lymph nodes 2/31, ileitis 2/31, tubal cysts 1/31, Meckel 1/31 diverticulum, several of the previous ones 10/31. Remission of symptoms: 30/55 (54.54%). In some cases, with partial improvement (4/55) or persistence of symptoms (21/55), organic and/ or psychological cause was demonstrated (16/25). CONCLUSIONS: Recurrent abdominal pain seems to have a significant association with an allergic or psychological history. Exploratory laparoscopy is a useful diagnostic and therapeutic technique.

INTRODUCCION Y OBJETIVOS: El dolor abdominal recurrente (DAR) supone > 3 episodios de dolor abdominal acompañados de afectación de la actividad diaria, durante > 3 meses. Nuestro objetivo es analizar el papel de la laparoscopia diagnóstica y/o terapéutica. MATERIAL Y METODOS: Estudio descriptivo, retrospectivo desde 2004 hasta 2016. Pacientes < 14 años con DAR a los que se les practicó laparoscopia. Variables: edad, sexo, antecedentes, hallazgos quirúrgicos, histología y evolución. RESULTADOS: 55 pacientes. Media de edad: 10,7 años. Mujeres 63, 6%. Probabilidad de comorbilidad alérgica: 27,27% [16,138- 40,962] (I.C 95%). Probabilidad de comorbilidad posterior psicológica: 12,72% [5,27 -24,48] (I.C 95%). Alteraciones histológicas 31/55 (56,36%): hiperplasia nodular linfoide 10/35, inflamación apendicular 7/31, fecalito 3/31, tumor carcinoide 1/31, fibrosis apendicular 3/31, divertículo de Meckel 1/31, asociación de varios de los anteriores 8/31. Alteraciones macroscópicas 31/55 (56,36%): patología apendicular 10/31, bridas 5/31, adenopatías 2/31, ileítis 2/31, quistes tubáricos 1/31, divertículo de Meckel 1/31, varios 10/31. Remisión: 30/ 55 (54,54%). En algunos casos con mejoría parcial (sin desaparición completa del dolor) (4/55) o persistencia de síntomas (21/55) se demostró causa orgánica y/o psicológica (16/25). CONCLUSIONES: El dolor abdominal recurrente parece presentar una asociación significativa con antecedentes alérgicos o psicológicos. La laparoscopia exploradora supone una técnica diagnóstica y terapéutica.

Use of Torulaspora delbrueckii and Saccharomyces cerevisiae in semi-industrial sequential inoculation to improve quality of Palomino and Chardonnay wines in warm climates.

AIMS: We have evaluated for the first time the impact of two commercial yeast strains (Torulaspora delbrueckii TD291 and Saccharomyces cerevisiae QA23) inoculated sequentially in musts of Chardonnay and Palomino Fino grape varieties grown under warm climate (South-west of Spain). METHODS AND RESULTS: Semi-industrial scale alcoholic fermentations (AF) were performed during the 2011 and 2012 harvests. Implantation analyses demonstrated that T. delbrueckii is the predominant strain until the end of the AF phase. Wines with sequential inoculation (SI) resulted in the production of low levels of acetic acid (which gives wine an undesirable 'vinegary' character), low acetaldehyde in Chardonnay and high in Palomino wines. The most salient attributes that contribute to the quality of the Chardonnay and Palomino wines produced were aroma intensity, fresh and tropical fruit character. CONCLUSIONS: This study demonstrated that SI of T. delbrueckii and S. cerevisiae contribute significantly to the improvement of Chardonnay wine aromas and the creation of new styles of wine for Palomino. SIGNIFICANCE AND IMPACT OF THE STUDY: This study has generated new knowledge about the biotechnological potential of T. delbrueckii (TD219) and S. cerevisiae (QA23) for improving the organoleptic properties of Chardonnay and Palomino wines.

[Home training as a treatment of faecal incontinence and sphincter dyssynergia]. / Entrenamiento anorrectal domiciliario como tratamiento de la encopresis y disinergia esfinteriana.

OBJECTIVE: The defecation disorders represents the 3% of consultations in pediatrics patients. Our goal was to demonstrate the effectiveness and efficiency of the reeducation of the defecation maneuver through home training in patients with encopresis and sphincter dyssynergia. MATERIAL AND METHODS: Study of patients with fecal incontinence treated with home training at our center between 2014-2015. Anorectal manometry was performed and was valued defecation maneuver by expulsion of rectal probe with or without the ball. Daily sessions were performed using a Foley catheter (18-20Fr.) with progressive filling of the balloon, maximum 20cc. The response to the treatment was assessed in terms of episodes of soiling. RESULTS: Seven patients (6 males and 1 female) with a mean age of 9.7 years (range, 5-15) were included; two patients with anorectal malformation history, 1 Hirschsprung disease, 1 sacrococcygeal teratoma and 3 functional encopresis. Three patients had soiling episodes daily and 4 patients frequently. The mean basal pressure of anal channel was 32.34mmHg (range, 11.74-50.75) with negative defecation maneuver in 2 cases, deficient in 3 and dyssynergic in 2 patients. The mean time of biofeedback therapy to be asymptomatic was 5.14 months (range, 2-11), with a mean of 16.14 months (range, 3-24), with the 7 patients currently maintained clean. CONCLUSIONS: The present study suggests that re-education of defecation maneuver through home training, seems to be an effective and efficient therapy, achieving excellent results in medium term.

OBJETIVO: Los trastornos de la defecación representan el 3% de las consultas en pediatría. Nuestro objetivo fue demostrar la eficacia y eficiencia de la reeducación en la maniobra defecatoria mediante el entrenamiento domiciliario en los pacientes con encopresis y disinergia esfinteriana. MATERIAL Y METODOS: Estudio de los pacientes con encopresis tratados mediante entrenamiento domiciliario en nuestro centro entre 2014-2015. Se realizó manometría anorrectal y fue valorada la maniobra defecatoria mediante la expulsión o no de la sonda rectal con o sin balón. Se realizaron sesiones diarias de forma domiciliaria empleando una sonda de Foley (18-20 Fr.) con llenado progresivo del balón, máximo 20 cc. Se evaluó la respuesta al tratamiento en función de la presencia de manchado. RESULTADOS: Se incluyeron 7 pacientes (6 varones y 1 mujer) con edad media de 9.7 años (rango, 5-15); dos con antecedente de malformación anorrectal, una enfermedad de Hirschsprung, 1 teratoma sacrococcígeo y 3 encopresis funcionales. Tres pacientes presentaban manchado diario y 4 de forma frecuente. La media de presión basal del canal fue de 32,34 mmHg (rango, 11,74-50,75) con maniobra defecatoria negativa en 2 casos, deficiente en 3 y disinérgica en 2. La media de tiempo realizando el entrenamiento intestinal hasta lograr estar asintomáticos fue 5.14 meses (rango, 2-11), con un seguimiento medio de 16,14 meses (rango, 3-24), manteniéndose limpios actualmente los 7 pacientes. CONCLUSIONES: El presente estudio sugiere que la reeducación de la maniobra defecatoria mediante entrenamiento domiciliario parece ser una terapia eficaz y eficiente, logrando excelentes resultados a medio plazo.

Gelled double emulsions as delivery systems for hydroxytyrosol and n-3 fatty acids in healthy pork patties.

Structuring of double emulsion offers the possibility to obtain a system with similar consistency to animal fat which is an interesting approach to improve the fat content of meat products. This article examines the suitability of gelled double emulsions (GDE) for use as a delivery system for n-3 PUFAs and hydroxytyrosol (Hxt) in pork patties. Effect of partial (MF/GDE sample) and total (LF/GDE) replacement of pork backfat with GDE with perilla oil (PO) as lipid phase and Hxt (in W1) on pork patty composition and properties was evaluated. Compared with the control sample (NF/CS), the products with PO contained less SFAs and higher proportions of LNA and ALA, and this difference augmented with the level of GDE. Addition of GDE increased Kramer shear force of cooked patties as the higher percentage of fat replaced was used. Oxidative stability of patties can be interpreted in terms of composition and structural factors, especially in relation with the use of GDE as a fat replacer. Products showed oxidation values greater than 1 after 3 days of refrigeration. NF/GDE and MF/GDE, with similar scores, were the most acceptable for the panellists. This technology is suitable for labelling meat products with nutritional and health claims.

Parent-reported prevalence of food allergy in Mexican schoolchildren: A population-based study.

BACKGROUND: Food allergy (FA) prevalence is well documented in developed countries and appears to be increasing, but remains unknown in most Latin American countries. We aimed to evaluate on a population basis the parent-reported prevalence of FA and its clinical characteristics in Mexican schoolchildren. METHODS: A validated Spanish version of a structured written questionnaire was administered to parents of schoolchildren aged 5-13 years old from Culiacan, Mexico. RESULTS: A total of 1049 parents responded to the survey (response rate, 84%). The estimated prevalence rates (95% CI) were: adverse food reactions 10.0% (8.3-11.9), "perceived FA, ever" 5.5% (4.3-7.0), "physician-diagnosed FA, ever" 4.9% (3.7-6.3), "immediate-type FA, ever" 4.4% (3.3-5.8), "immediate-type FA, current" 3.5% (2.6-4.8), and anaphylaxis 1.2% (0.72-2.1). Immediate hypersensitivity reactions were mainly triggered by the consumption of shrimp (1.3%), other shellfish (0.7%), strawberry (0.6%), chocolate (0.5%), and egg (0.4%). Schoolchildren with "immediate-type FA, current" had more atopic dermatitis and allergic rhinitis (p<0.05), but not asthma or drug allergy (p>0.05) than children without FA. All cases of anaphylaxis sought medical attention, but only one child had physician-diagnosed anaphylaxis and was advised to acquire an epinephrine autoinjector. CONCLUSIONS: The prevalence of "immediate-type FA, current" to any food is 3.5% in Mexican schoolchildren. The poor recognition of anaphylaxis and the low frequency of prescription of epinephrine autoinjectors suggest that acute food-induced allergic reactions are not optimally managed in Mexico.

Impact of harmful use of alcohol on the sedation of critical patients on mechanical ventilation: A multicentre prospective, observational study in 8 Spanish intensive care units.

PURPOSE: To evaluate the impact of a history of harmful use of alcohol (HUA) on sedoanalgesia practices and outcomes in patients on mechanical ventilation (MV). METHODS: A prospective, observational multicentre study was made of all adults consecutively admitted during 30 days to 8 Spanish ICUs. Patients on MV >24h were followed-up on until discharge from the ICU or death. Data on HUA, smoking, the use of illegal (IP) and medically prescribed psychotropics (MPP), sedoanalgesia practices and their related complications (sedative failure [SF] and sedative withdrawal [SW]), as well as outcome, were prospectively recorded. RESULTS: A total of 23.4% (119/509) of the admitted patients received MV >24h; 68.9% were males; age 57.0 (17.9) years; APACHE II score 18.8 (7.2); with a medical cause of admission in 53.9%. Half of them consumed at least one psychotropic agent (smoking 27.7%, HUA 25.2%; MPP 9.2%; and IP 7.6%). HUA patients more frequently required PS (86.7% vs. 64%; p<0.02) and the use of >2 sedatives (56.7% vs. 28.1%; p<0.02). HUA was associated to an eightfold (p<0.001) and fourfold (p<0.02) increase in SF and SW, respectively. In turn, the duration of MV and the stay in the ICU was increased by 151h (p<0.02) and 4.4 days (p<0.02), respectively, when compared with the non-HUA group. No differences were found in terms of mortality. CONCLUSIONS: HUA may be associated to a higher risk of SF and WS, and can prolong MV and the duration of stay in the ICU in critical patients. Early identification could allow the implementation of specific sedation strategies aimed at preventing these complications.

Less superior adjacent syndrome and lower reoperation rate. Medium- and long-term results of cervical arthroplasty versus anterior cervical arthrodesis: Systematic review and meta-analysis of randomized clinical trials. / Menor síndrome adyacente superior y menor tasa de reoperación. Resultados a mediano y largo plazo de la artroplastia cervical frente a la artrodesis cervical anterior: revisión sistemática y metaanálisis de ensayos clínicos aleatorizados.

OBJECTIVE: To compare medium- and long-term postoperative surgical results, especially the adjacent syndrome rate, adverse event rate, and reoperation rate, of patients operated on with cervical arthroplasty or anterior cervical arthrodesis in published randomized clinical trials (RCTs), at one cervical level. METHODS: Systematic review and meta-analysis. Thirteen RCTs were selected. The clinical, radiological and surgical results were analyzed, taking the adjacent syndrome rate and the reoperation rate as the primary objective of the study. RESULTS: Two thousand nine hundred and sixty three patients were analyzed. The cervical arthroplasty group showed a lower rate of superior adjacent syndrome (P<0.001), lower reoperation rate (P<0.001), less radicular pain (P=0.002), and a better score of neck disability index (P=0.02) and SF-36 physical component (P=0.01). No significant differences were found in the lower adjacent syndrome rate, adverse event rate, neck pain scale, or SF-36 mental component. A range of motion of 7.91 degrees was also found at final follow-up, and a heterotopic ossification rate of 9.67% in patients with cervical arthroplasty. CONCLUSION: In the medium and long-term follow-up, cervical arthroplasty showed a lower rate of superior adjacent syndrome and a lower rate of reoperation. No statistically significant differences were found in the rate of inferior adjacent syndrome or in the rate of adverse events.

[Translated article] Less superior adjacent syndrome and lower reoperation rate. Medium- and long-term results of cervical arthroplasty versus anterior cervical arthrodesis: Systematic review and meta-analysis of randomized clinical trials.

OBJECTIVE: To compare medium- and long-term postoperative surgical results, especially the adjacent syndrome rate, adverse event rate, and reoperation rate, of patients operated on with cervical arthroplasty or anterior cervical arthrodesis in published randomized clinical trials (RCTs), at one cervical level. METHODS: Systematic review and meta-analysis. Thirteen RCTs were selected. The clinical, radiological and surgical results were analyzed, taking the adjacent syndrome rate and the reoperation rate as the primary objective of the study. RESULTS: Two thousand nine hundred and sixty three patients were analyzed. The cervical arthroplasty group showed a lower rate of superior adjacent syndrome (P<0.001), lower reoperation rate (P<0.001), less radicular pain (P=0.002), and a better score of neck disability index (P=0.02) and SF-36 physical component (P=0.01). No significant differences were found in the lower adjacent syndrome rate, adverse event rate, neck pain scale, or SF-36 mental component. A range of motion of 7.91° was also found at final follow-up, and a heterotopic ossification rate of 9.67% in patients with cervical arthroplasty. CONCLUSION: In the medium and long-term follow-up, cervical arthroplasty showed a lower rate of superior adjacent syndrome and a lower rate of reoperation. No statistically significant differences were found in the rate of inferior adjacent syndrome or in the rate of adverse events.

Response to azacitidine in patients with chronic myelomonocytic leukemia according to overlap myelodysplastic/myeloproliferative neoplasms criteria.

BACKGROUND: Azacitidine (AZA) is approved for the treatment of high-risk chronic myelomonocytic leukemia (CMML) of myelodysplastic (MD) subtype. Data of response rates using the specific response criteria for this disease are scarce. The aim of this study was to evaluate the response to AZA in patients diagnosed with CMML from the Spanish Registry of Myelodysplastic Syndromes (MDS) applying the overlap myelodysplastic/myeloproliferative neoplasms (MDS/MPN) response criteria. METHODS: We retrospectively studied 91 patients with CMML treated with at least one cycle of AZA from the Spanish Registry of MDS. As it was a real-world study, the response rate was evaluated between cycle 4 and 6, applying the MDS/MPN response criteria FINDINGS: The overall response rate at cycle 4-6 was 58%. Almost half of the patients achieved transfusion independence and one quarter showed clinical benefit, regardless of the CMML French-American-British (FAB) and World Health Organization (WHO) subtypes and CMML Specific Prognosis Scoring (CPSS) risk groups. Toxicity was higher in the MD-CMML subtype. INTERPRETATION: In our series, most CMML patients achieved an overall response rate with AZA according to the overlap-MDS/MPN response criteria regardless of the CMML FAB and WHO subtypes and CPSS risk groups. Thus, AZA may also be a treatment option for patients with the myeloproliferative CMML subtype and those with a lower-risk CPSS, but symptomatic.

Different methylation signatures at diagnosis in patients with high-risk myelodysplastic syndromes and secondary acute myeloid leukemia predict azacitidine response and longer survival.

BACKGROUND: Epigenetic therapy, using hypomethylating agents (HMA), is known to be effective in the treatment of high-risk myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) patients who are not suitable for intensive chemotherapy and/or allogeneic stem cell transplantation. However, response rates to HMA are low and there is an unmet need in finding prognostic and predictive biomarkers of treatment response and overall survival. We performed global methylation analysis of 75 patients with high-risk MDS and secondary AML who were included in CETLAM SMD-09 protocol, in which patients received HMA or intensive treatment according to age, comorbidities and cytogenetic. RESULTS: Unsupervised analysis of global methylation pattern at diagnosis did not allow patients to be differentiated according to the cytological subtype, cytogenetic groups, treatment response or patient outcome. However, after a supervised analysis we found a methylation signature defined by 200 probes, which allowed differentiating between patients responding and non-responding to azacitidine (AZA) treatment and a different methylation pattern also defined by 200 probes that allowed to differentiate patients according to their survival. On studying follow-up samples, we confirmed that AZA decreases global DNA methylation, but in our cohort the degree of methylation decrease did not correlate with the type of response. The methylation signature detected at diagnosis was not useful in treated samples to distinguish patients who were going to relapse or progress. CONCLUSIONS: Our findings suggest that in a subset of specific CpGs, altered DNA methylation patterns at diagnosis may be useful as a biomarker for predicting AZA response and survival.

Smartphone addiction, risk factors and its adverse effects in nursing students: A systematic review and meta-analysis.

BACKGROUND: Smartphones are technological devices that have a great impact on people's daily lives changing their habits and behaviors. The utilities and capabilities of these devices are increasing and the foresight is that this tendency will grow in the next years. However, the problematic use of the smartphone has increased dangerously, interfering with the clinical practice of healthcare professionals. OBJECTIVE: To conduct a systematic review and a meta-analysis to estimate the prevalence of smartphone addiction, also known as nomophobia, in nursing students and to identify its related factors and its negative effects. DESIGN: Systematic Review and Meta-analysis. DATA SOURCES: The literature search was done in CINAHL, Proquest, Pubmed, Scopus and Web of Science databases. REVIEW METHODS: The search equation was "(nomophobia OR smartphone) AND nurs* student*". n = 16 articles were identified. RESULTS: The meta-analytic estimation of nomophobia or smartphone addiction was 22% (CI95% 18%-26%] in a sample of n = 2780 nursing students. Smartphone usability is very extended during the clinical practice by nursing students. They indicated using the device or watching other students distracted with their smartphone. Some variables related to excessive use in nursing students are lower sleep quality, lower self-esteem, higher social distress, lower perceived social support or lower communication skills between others. CONCLUSIONS: The main use of the smartphone by nursing students is for communication with other people and some of them indicate that it can be a distraction.

[Relationship between the radiological length of the opaque enema and the aganglionic length of the segment in Hirschsprung's disease]. / Relación entre la longitud radiológica del enema opaco y la longitud agangliónica de la pieza en la enfermedad de Hirschsprung.

INTRODUCTION: Barium enema was the first method used for the diagnosis of Hirschsprung's disease, with the appearance of anorectal manometry and its combination with rectal suction biopsy, barium enema has lost value as a diagnosis method but it has also gain importance to decide the surgical technique that will be used for the correction of the disease. AIM: To determine the correlation between the length of the affected segment showed by barium enema valued and the length of the removed piece. MATERIALS AND METHODS: We have studied all Hirschsprung disease's cases diagnosed and treated in our center since 1998, 127 patients underwent Soave-Boley's technique and 51 De La Torre's descent technique. Routinely preoperative barium enema was performed in all cases and determine the location of the transition zone and compared with the aganglionic segment's length specified by pathologist. RESULTS: At 90% of cases the transition zone could be seen at barium enema, it's most common location was rectosigmoid. After statistically analysis the length measured at radiology tests and the length of the anatomic piece showed a low correlation (kappa index 0.0159), being highest values at rectosigmoid transition and very low values at long affected segments. CONCLUSIONS: Barium enema in Hirschsprung's disease is valuable to decide the best surgical technique in each singular case but not to determine the exact length of affected segments. In case of a aganglionic long-segment suspicion, biopsies may be necessary to determinate preoperative length of affected segments.

Perioperative anesthetic management of patients with malignant pleural mesothelioma undergoing cytoreductive surgery and intraoperative chemotherapy. / Manejo anestésico perioperatorio de pacientes con mesotelioma maligno pleural intervenidos mediante cirugía citorreductora y quimioterapia intratorácica.

INTRODUCTION: Cytoreductive surgery with hyperthermic intraoperative chemotherapy (HITHOC) is a therapeutic option for treatment of malignant pleural mesothelioma. Anesthetic management might be challenging. PATIENTS AND METHODS: A descriptive analysis of a case series is presented. Seven patients with malignant pleural mesothelioma diagnostic undergoing HITHOC surgery were studied. Combined general and epidural anesthesia were administered. An intensive hemodynamic monitorization was implemented. Data regarding perioperative course was analyzed. RESULTS: Between May 2015 and October 2018 7patients underwent HITHOC procedure. Blood transfusions were administered in all patients, and 5of the 7patients required vasoactive drug administration. Extubation at the end of the procedure was able in 6of the 7patients. The median length of stay in ICU was 4 days, and 29 days for the whole hospitalary stay. No significant postoperative pain was observed. CONCLUSIONS: HITHOC surgery is a complex procedure in which several hemodynamic changes occur. An intensive intraoperative monitorization was useful for controlling complications.

A regulatory cascade involving retinoic acid, Cbfa1, and matrix metalloproteinases is coupled to the development of a process of perichondrial invasion and osteogenic differentiation during bone formation.

Tissue-remodeling processes are largely mediated by members of the matrix metalloproteinase (MMP) family of endopeptidases whose expression is strictly controlled both spatially and temporally. In this article, we have examined the molecular mechanisms that could contribute to modulate the expression of MMPs like collagenase-3 and MT1-MMP during bone formation. We have found that all-trans retinoic acid (RA), which usually downregulates MMPs, strongly induces collagenase-3 expression in cultures of embryonic metatarsal cartilage rudiments and in chondrocytic cells. This effect is dose and time dependent, requires the de novo synthesis of proteins, and is mediated by RAR-RXR heterodimers. Analysis of the signal transduction mechanisms underlying the upregulating effect of RA on collagenase-3 expression demonstrated that this factor acts through a signaling pathway involving p38 mitogen-activated protein kinase. RA treatment of chondrocytic cells also induces the production of MT1-MMP, a membrane-bound metalloproteinase essential for skeletal formation, which participates in a proteolytic cascade with collagenase-3. The production of these MMPs is concomitant with the development of an RA-induced differentiation program characterized by formation of a mineralized bone matrix, downregulation of chondrocyte markers like type II collagen, and upregulation of osteoblastic markers such as osteocalcin. These effects are attenuated in metatarsal rudiments in which RA induces the invasion of perichondrial osteogenic cells from the perichondrium into the cartilage rudiment. RA treatment also resulted in the upregulation of Cbfa1, a transcription factor responsible for collagenase-3 and osteocalcin induction in osteoblastic cells. The dynamics of Cbfa1, MMPs, and osteocalcin expression is consistent with the fact that these genes could be part of a regulatory cascade initiated by RA and leading to the induction of Cbfa1, which in turn would upregulate the expression of some of their target genes like collagenase-3 and osteocalcin.

Induction of hepatic aminolevulinate acid synthetase activity by isoflurane in a genetic model for erythropoietic protoporphyria.

Erythropoietic Protoporphyria (EPP) is an inherited deficiency of ferrochelatase, the last enzyme of the heme pathway. Under general anaesthesia, some patients develop neurological dysfunction suggesting upregulation in heme biosynthesis similar to that described for acute porphyrias after xenobiotic administration. Our aim has been to evaluate whether Isoflurane induces alterations in the heme pathway in a mouse model for EPP. Administration of Isoflurane (a single dose of 2 ml/kg, i.p) to wild-type (+/+), heterozygous (+/Fechm1Pas) and homozygous (Fechm1Pas/Fechm1Pas) mice, was evaluated by measuring the activity of delta-aminolevulinic acid synthetase (ALA-S) and Porphobilinogen-deaminase (PBG-D) in different tissues, as well as Heme oxygenase (HO), cytochrome P-450, CYP2E1 and glutathione levels in liver. Porphyrin precursors were measured in 24 h-urine samples. Fechm1Pas/Fechm1Pas mice receiving anaesthesia show enhanced ALA-S and CYP2E1 activities in the liver and increased urinary excretion of porphyrin precursors. No alterations were found in either PBG-D or HO activities. Diminished glutathione levels suggest that anaesthesia may produce oxidative stress in these animals. In conclusion, Isoflurane induces ALA-S activity and increased excretion of porphyrin precursors in EPP mice. These findings appear to confirm our previous hypothesis and indicate that Isoflurane may be an unsafe anaesthetic not only for patients with acute porphyrias but also for individuals with non acute porphyrias.

Identification and characterization of HMBS gene mutations in Spanish patients with acute intermittent porphyria.

Acute intermittent porphyria (AIP), the most common acute hepatic porphyria, is an autosomal dominant disorder with low penetrance that results from a partial deficiency of hydroxymethylbilane synthase (HMBS), the third enzyme in the heme biosynthetic pathway. The disease is clinically characterized by acute neurovisceral attacks that are precipitated by several factors including certain drugs, steroid hormones, alcohol and fasting. Early diagnosis and counselling are essential to prevent attacks, being mutation analysis the most reliable method to identify asymptomatic carriers in AIP families. In this study we have investigated the molecular defect in 15 unrelated Spanish AIP patients. Mutation analysis of the HMBS gene revealed a total of fourteen mutations including six novel ones, two of them were on the same allele in one patient. The novel mutations were three missense (R26L, R173G and D178H), two frameshift (c.749_765dup and c.874insC) and one intronic deletion (IVS12+3_+11delAGGGCCTGT). RT-PCR and sequencing demonstrated that the intronic mutation caused abnormal splicing and exon 12 skipping. Prokaryotic expression of the novel missense mutations showed that only D178H had significant residual activity. These findings will facilitate the accurate identification of presymptomatic AIP carriers in these families and they further emphasize the molecular heterogeneity of AIP in Spain.

Localized acquired cutis laxa secondary to interstitial granulomatous dermatitis.

We report the case of a 68-year-old woman who had interstitial granulomatous dermatitis associated with seronegative polyarthritis. Two years later, this had evolved to become localized acquired cutis laxa.

[Incidence of metachronic contralateral inguinal hernia]. / Incidencia de la hernia inguinal contralateral metácrona.

INTRODUCTION: There are many observational studies about the incidence of metachronous contralateral inguinal hernia (MIH). Metaanalysis allows to resume the results of individual studies in one with a higher level of evidence. AIM: to know the incidence of MIH in order to decide the contralateral exploration. MATERIAL AND METHODS: We do an extensive bibliographic review in Medline, Embase and Cochrane Central. Data analysis is done with RevMan 4.2. RESULTS: 632 abstracts were screened by only one revisor who selected 27 studies: 17802 inguinal hernias and 1209 MIH. Follow up is between 6 months and 10 years. Due to high heterogeneity, a randomized effect analysis (Der Simonian and Lard) is done. The incidence of MIH is 6,96% (6,07-7,85); 14 hernia must be operated to avoid one MIH (NNT). If the original side of the hernia is left, the probability of MIH is 1.81 times higher (NNT =10). The secondary analysis indicates that there is no important publication bias. CONCLUSIONS: Rutinary bilateral inguinal exploration is not justified. Only in high anaesthetic risk patients, bilateral exploration should be suggested.

[Results of the percutaneous retrograde embolization as the first choice in the treatment of varicocele]. / Resultados de la embolización retrógrada percutánea como primera opción en el tratamiento del varicocele.

There is evidence that early varicocele treatment decreases testicular damage. The minimally invasive techniques such as laparoscopic varicocelectomy, antegrade sclerotherapy and percutaneous retrograde embolisation, are acquiring greater significance in the treatment of this disease. Since 1994, a series of 51 children, aged 7-16 years (mean, 12.9 years), with left-sided varicocele grades 2 (47%) and 3 (53%) were treated in our institution by percutaneous retrograde embolisation using coils. The right basilica vein was the most widely used (70%) followed by the right femoral vein. Seven children (13.7%) had complications: perforation of internal spermatic vein was the most frequent and treated conservatively. No child presented hydrocele after radiological procedures. Embolisation was accomplished succesfully at the first attempt in 35 (68.6%) of the 51 children and in 45 (88.2%) after a second embolisation. The follow-up ranges from 7 months to 5 ? years (mean, 1.8 years). The patients were monitored with clinical and doppler ultrasound examination 3, 6 and 12 months after the treatment. Retrograde embolisation is a safe and efficient minimally invasive treatment for correcting varicoceles in children. We performed retrograde embolisation as first choice of varicocele treatment in children; a second embolisation or conventional surgery for primary failure or late recurrence should be considered.