Significant heterogeneity in the diagnosis and long-term management of Wilson disease: Results from a large multicenter Spanish study.

There is uncertainty regarding Wilson's disease (WD) management. OBJECTIVES: To assess, in a multicenter Spanish retrospective cohort study, whether the approach to WD is homogeneous among centers. METHODS: Data on WD patients followed at 32 Spanish hospitals were collected. RESULTS: 153 cases, 58% men, 20.6 years at diagnosis, 69.1% hepatic presentation, were followed for 15.5 years. Discordant results in non-invasive laboratory parameters were present in 39.8%. Intrahepatic copper concentration was pathologic in 82.4%. Genetic testing was only done in 56.6% with positive results in 83.9%. A definite WD diagnosis (Leipzig score ≥4) was retrospectively confirmed in 92.5% of cases. Chelating agents were standard initial therapy (75.2%) with frequent modifications (57%), particularly to maintenance zinc. Enzyme normalization was not achieved by one third, most commonly in the setting of poor compliance, lack of genetic mutations and/or presence of cardiometabolic risk factors. Although not statistically significant, there were trends for sex differences in number of diagnosed cases, age at diagnosis and biochemical response. CONCLUSIONS: Significant heterogeneity in diagnosis and management of WD patients emerges from this multicenter study that includes both small and large reference centers. The incorporation of genetic testing will likely improve diagnosis. Sex differences need to be further explored.

The ALK translocation in advanced non-small-cell lung carcinomas: preapproval testing experience at a single cancer centre.

AIMS: To study the ALK translocation in patients with advanced non-small-cell lung carcinomas (NSCLCs) seen at a European cancer centre, and its association with EGFR mutations, KRAS mutations and MET amplification. METHODS AND RESULTS: The study included samples from 86 patients diagnosed with advanced NSCLC. ALK fluorescence in-situ hybridization (FISH) was performed using the ALK break-apart probe set (Vysis). ALK FISH-positive cases were defined as those with more than 15% break-apart signals or isolated red signals in 50 cells. EGFR and KRAS mutations were determined by direct sequencing. All ALK-positive cases were analysed retrospectively for MET amplification using a FISH assay, and for ALK mutations by sequencing. We found nine (10.5%) ALK-positive cases, all in adenocarcinomas and the majority in female patients (88.9%). Signet ring cells were observed in four (44.4%) of the nine patients. None of the ALK translocated cases showed MET amplifications or EGFR, KRAS and ALK mutations. CONCLUSIONS: The prevalence of ALK translocation in an unselected population of European patients with advanced NSCLCs was 10%. This alteration was mutually exclusive with EGFR and KRAS mutations, as well as with MET amplification. If multiplexing is considered at the preanalytical phase, lung biopsy specimens are sufficient for performing several predictive assays.

Pyrolysis-induced phosphorus transformations for biosolids from diverse sources.

Biosolids have been long used as a soil amendment to promote nutrient recovery. The readily releasable forms of nutrients present in this biowaste, such as phosphorus (P), along with their over application, can be detrimental to the environment, causing eutrophication. Pyrolysis, the thermal decomposition of organic materials at elevated temperature and low oxygen, seems to be a promising strategy to lower P release from biowastes such as biosolids. We pyrolyzed biosolids from various treatments and locations (Florida and Illinois; Galicia, Spain; and São Paulo, Brazil) to convert to biochar. Our objectives were (a) to use solid-state assessments, such as X-ray diffraction and scanning electron microscopy, and chemical assessments, such as water-soluble P (WSP), pH, Mehlich 3-extractable P (M3-P), total P (TP), and total Kjeldahl nitrogen, to evaluate changes caused by the conversion and (b) to compare P leaching potentials of biosolids and their corresponding biochars on two soils with varying P retention capacities. Pairwise comparisons indicated that biochar conversion significantly increased TP in the final material, but the absolute WSP decreased. However, M3-P remained the same after conversion to biochar. Cumulative P leached as a fraction of TP was greater for biosolids than their corresponding biochars. Two soils with contrasting P retention capacities predictably differed in P leaching behaviors as amended with biosolids and biochars. Differences suggest that future research could evaluate the efficacy of using mixtures of biosolids and biochar for a given soil to maintain soil fertility while reducing environmental P loss risk.

Silvopasture policy promotion in European Mediterranean areas.

Silvopasture is the deliberate integration of a woody component with grazed pastures as understorey. It is one of the most extended agroforestry practices all over the world. Silvopasture use is key to increase the sustainability of livestock farming systems as silvopasture reduces the use of concentrates since the woody component provides feed for animals. However, it is not an extensively used practice in Europe. This paper aims at evaluating, from Eurostat, LUCAS database and the 118 rural development programs, the current situation of permanent grasslands in the Mediterranean area of Europe as well as the rural development programmes fostering silvopasture to better understand how sustainable land use systems are promoted and provide insights to foster silvopasture across Europe. The results of this study show that most of the policy measures related to silvopasture are adapted to the local necessity. The already existing agroforestry managed land (dehesas/montado) are related to measures supporting regeneration and maintenance while in those areas where agroforestry does not exist the measures are related to forest fire prevention.

Experience with GyneFIX insertions in Spain: favorable acceptance of the intrauterine contraceptive implant with some limitations.

This study evaluates the incidents associated with GyneFIX insertion and first-year expulsion and continuity rates within the usual intrauterine contraceptive practice of a working group of Spanish professionals (GESEG), formed specifically with this aim. It is a prospective, multicenter, observational study of GyneFIX insertion in 1684 women. Data were prospectively collected on a structured form and processed centrally. Interest was focused on difficulties encountered during the insertion procedure and symptoms experienced during insertion. All terms were defined by consensus. Among the total, 18.6% of the women were nulliparous. GyneFIX insertion was rated easy in 92%, with more difficulty in nulliparous women, who showed significantly more symptoms during insertion of the device. First-year expulsion and continuity rates were 5.6 and 88 per 100 women, respectively. The pregnancy rate was 0.3 per 100 women/years. The GyneFIX system is an interesting alternative to standard IUDs for intrauterine contraception with copper, particularly in women who have experienced expulsion of other types of IUDs. Experienced professionals in IUD insertion quickly acquire familiarity with the GyneFIX insertion system, but proper implantation does not completely eliminate the risk of expulsion. Thus, the insertion system should be further modified to achieve a simpler, safer technique.

Sewage sludge fertiliser use: implications for soil and plant copper evolution in forest and agronomic soils.

Fertilisation with sewage sludge may lead to crop toxicity and environmental degradation. This study aims to evaluate the effects of two types of soils (forest and agronomic), two types of vegetation (unsown (coming from soil seed bank) and sown), and two types of fertilisation (sludge fertilisation and mineral fertilisation, with a no fertiliser control) in afforested and treeless swards and in sown and unsown forestlands on the total and available Cu concentration in soil, the leaching of this element and the Cu levels in plant. The experimental design was completely randomised with nine treatments and three replicates. Fertilisation with sewage sludge increased the concentration of Cu in soil and plant, but the soil values never exceeded the maximum set by Spanish regulations. Sewage sludge inputs increased both the total and Mehlich 3 Cu concentrations in agronomic soils and the Cu levels in plant developed in agronomic and forest soils, with this effect pronounced in the unsown swards of forest soils. Therefore, the use of high quality sewage sludge as fertiliser may improve the global productivity of forest, agronomic and silvopastoral systems without creating environmental hazards.

[Effectiveness of a therapeutic practice on overweight in primary care]. / Eficacia de una intervención terapéutica sobre el exceso de peso en atención primaria.

OBJECTIVES: To evaluate the effectiveness of an intervention program on overweight to achieve a minimum decrease of 5% or a optimum of 10% of initial weight a year, and to identify the factors associated with slimming. DESIGN: Randomized controlled trial. SETTING: Urban health centre. PARTICIPANTS: Overweight subjects over 18 years old (n=169), wanting treatment or referred by their doctor. They were randomly assigned to the intervention (n=95) or control (n=70) group. INTERVENTIONS: Intervention group: yearly program with consultations every 15-21 days, using a low calorie diet, prescribing physical exercise, changing of habits and health education. CONTROL GROUP: low calorie diet, seen 3 times/year. RESULTS: The minimum weight loss was achieved in 6 months by 37.9% of the intervention group and by 21.4% of the control group. At one year, 45.3% of the intervention subjects and 24.3% of the control group reached this objective. The optimum loss of 10% was higher in the intervention group, but was not statistically significant. The minimum objective was associated with the intervention group (OR=4.5 [1.36-14.49]). It was also associated to the number of visits during therapy (OR=1.85 [1.50-2.29]) and diabetes (OR=10 [1.13-90.9]). CONCLUSIONS: The intervention group had a loss of 5% of initial weight in the first 6 months, 16.5% more than the control group, and 21% more in one year. Achieving the minimum objective was associated with the intervention group, the number of visits and the diagnosis of diabetes.

Pleural aspergillosis with bronchopleurocutaneous fistula and costal bone destruction: a case report.

A 65-year-old man who, when young, had had tuberculosis treated by therapeutic pneumothorax, consulted his family physician for a constitutional syndrome and dyspnea. At this time radiologic studies showed left pleural effusion with bilateral calcified plaques, an infiltrate in the upper left lobe, and a picture compatible with aspergilloma, all suggesting semi-invasive aspergillosis. The patient failed to show up for his followup visit, so no therapy could be started or further diagnostic tests ordered. One month later he was admitted to this hospital for a bronchopleural fistula (empyema necessitatis) with subsequent spontaneous hydropneumothorax and costal bone involvement. The patient underwent surgery because of his rapid worsening condition. Biopsy examination revealed a large pleural aspergilloma. Despite immediate antifungal therapy, the patient died. We believe this to be the first report of pleural Aspergillus with a bronchopleurocutaneous fistula and costal bone destruction.