“Western Mediterranean” (WM) broad band network: permanent seismic monitoring at the Ibero-Maghrebian region

The “Ibero-Maghrebian” region, a western part of the Eurasia-Africa plate boundary, is a broad deformation area without a well defined plate boundary line, comprising the Southern part of the Iberian Peninsula, the Northwestern part of Africa, the Gulf of Cadiz and the Alboran Sea, with a convergence between Iberia and Africa at some mm/year (Nuvel-1A model, DeMets et al, 1994) in a NNW-SSE direction, and a dif- ferential motion at Alboran Sea area (Buforn et al, 1995). Although seismic activity is characterized by a low to moderate magnitude and shallow depth earthquakes, big events are also well documented, among others: Lisbon earthquake (1755, I0= X), Gulf of Cadiz (1969, Ms= 8.1), or, more recently Bourmedes (2003, Mw= 7.1), some them with an associated tsunami, like Lisbon or Bourmedes earthquakes. An interme- diate seismic activity is also clearly registered from Gulf of Cadiz to mid Alboran Sea (Martin Davila and Pazos, 2003), and very deep earthquakes (h> 650 km) have been registered at Granada basin (1954, M= 7.0). In order to better study the seismic characteristics of this area, from 1996 on, ROA and the University Complutense of Madrid (UCM), with the collaboration of Geo- forschungZentrum of Potsdam (GFZ), have deployed a broad band seismic net with stations located at Southern Spain and Spanish possessions located Northern Africa, mainly surrounding the Alboran Sea, complementing the previously installed ROA SP and LP stations. This net has been named as “Western Mediterranean net” (WM FDSN code). As net was evolving, new Institutes and stations joined WM net, such a way at present is formed by ten stations located at: San Fernando (SFS), Málaga (MALA), Cartagena (CART), and Evora (UEVO, University of Evora, Portugal) at Iberian peninsula, Mahón (MAHO) at Minorca island, three stations at Melilla (MELI), Peñón de Vélez-Gomera (PVLZ), and Ceuta (CEU) at Spanish villages located Northern Africa in South Alboran Sea area, and Averroes (AVE) and Ifrane (IFR) installed at Morocco mainland as a joined effort among ISRABAT (Institut Scientifique, Univer- sité Mohammed V), ROA and UCM. Most stations are collocated with permanent geodetic GPS stations (Gárate et al, 2004). Next future plans include the installation of five stations by U. Evora at Southern Portugal area, a new station at Morocco by ISRABAT, ROA and UCM and also a new station at Oran (Algeria), as a collaboration among Université d’Oran (Algeria), ROA and UCM. All them will be associated to WM net. All WM network stations include Streckeisen STS-2 sensor, a Quanterra or Earth Data digitizer, and a SeiscomP process system (Heinloo, 2004), and all them are available in real time via phone modem or Internet, except PVLZ and CEU, which will be in short. In this work, partly funded by the Spanish Ministry of Education and Science (MEC) through the project REN2006-10311-C03-01/02 (RISTE), we will present the present status, the next future plans and some related activities of WM net

Receiver function study of the Hellenic subduction zone: imaging crustal thickness variations and the oceanic Moho of the descending African lithosphere

We use data from recently installed broad-band seismographs on the islands of Crete, Gavdos, Santorini, Naxos and Samos in the Hellenic subduction zone to construct receiver function images of the crust and upper mantle from south of Crete into the Aegean Sea. The stations are equipped with STS-2 seismometers and they are operated by GFZ Potsdam, University of Chania and ETH Zürich. Teleseismic earthquakes recorded by these stations at epicentral distances between 35° and 95° have been used to calculate receiver functions. The receiver function method is a routinely used tool to detect crustal and upper-mantle discontinuities beneath a seismic station by isolating the P-S converted waves from the coda of the P wave. Converted P-S energy from the oceanic Moho of the subducted African Plate is clearly observed beneath Gavdos and Crete at a depth ranging from 44 to 69 km. This boundary continues to the north to nearly 100 km depth beneath Santorini island. Because of a lack of data the correlation of this phase is uncertain north of Santorini beneath the Aegean Sea. Moho depths were calculated from primary converted waves and multiply reflected waves between the Moho and the Earth's surface. Beneath southern and eastern Crete the Moho lies between 31 and 34 km depth. Beneath western and northern Crete the Moho is located at 32 and 39 km depth, respectively, and behaves as a reversed crust-mantle velocity contrast, possibly caused by hydration and serpentinization of the forearc mantle peridotite. The Moho beneath Gavdos island located south of Crete in the Libyan Sea is at 26 km depth, indicating that the crust south of the Crete microcontinent is also thinning towards the Mediterranean ridge. This makes it unlikely that part of the crust in Crete consists of accreted sediments transported there during the present-day subduction process which began approximately 15 Ma because the backstop, i.e. the boundary between the current accretionary wedge of the Mediterranean ridge and the Crete microcontinent, is located approximately 100 km south of Gavdos. A seismic boundary at 32 km depth beneath Santorini island probably marks the crustal base of the Crete microcontinent. A shallower seismic interface beneath Santorini at 20-25 km depth may mark the depth of the detachment between the Crete microcontinent and the overlying Aegean subplate. The Moho in the central and northern Aegean, at Naxos and Samos, is observed at 25 and 28 km depth, respectively. Assuming a stretching factor of 1.2-1.3, crustal thickness in the Aegean was 30-35 km at the inception of the extensional regime in the Middle Miocen

Brucellosis in Humans and Animals in Kyrgyzstan

Brucellosis is a globally reemerging and neglected zoonosis causing serious public health problems as well as considerable economic losses due to infection of livestock. Although the epidemiology of brucellosis has been well studied and its various aspects in humans and animals are well understood, it is still one of the most challenging health problems in many developing countries such as Kyrgyzstan. This review describes epidemiological characteristics of brucellosis in humans and animals, its impact on animal production and the role of implemented infection control measures in Kyrgyzstan. Particularly, introduction of mass vaccination in small ruminants evidently contributed to control of brucellosis in Kyrgyzstan, reducing the number of infections in animals as well as humans

Microcephaly with simplified gyration, epilepsy, and infantile diabetes linked to inappropriate apoptosis of neural progenitors

We describe a syndrome of primary microcephaly with simplified gyral pattern in combination with severe infantile epileptic encephalopathy and early-onset permanent diabetes in two unrelated consanguineous families with at least three affected children. Linkage analysis revealed a region on chromosome 18 with a significant LOD score of 4.3. In this area, two homozygous nonconserved missense mutations in immediate early response 3 interacting protein 1 (IER3IP1) were found in patients from both families. IER3IP1 is highly expressed in the fetal brain cortex and fetal pancreas and is thought to be involved in endoplasmic reticulum stress response. We reported one of these families previously in a paper on Wolcott-Rallison syndrome (WRS). WRS is characterized by increased apoptotic cell death as part of an uncontrolled unfolded protein response. Increased apoptosis has been shown to be a cause of microcephaly in animal models. An autopsy specimen from one patient showed increased apoptosis in the cerebral cortex and pancreas beta cells, implicating premature cell death as the pathogenetic mechanism. Both patient fibroblasts and control fibroblasts treated with siRNA specific for IER3IP1 showed an increased susceptibility to apoptotic cell death under stress conditions in comparison to controls. This directly implicates IER3IP1 in the regulation of cell survival. Identification of IER3IP1 mutations sheds light on the mechanisms of brain development and on the pathogenesis of infantile epilepsy and early-onset permanent diabetes.Facultad de Ciencias Médica

Microcephaly with simplified gyration, epilepsy, and infantile diabetes linked to inappropriate apoptosis of neural progenitors

We describe a syndrome of primary microcephaly with simplified gyral pattern in combination with severe infantile epileptic encephalopathy and early-onset permanent diabetes in two unrelated consanguineous families with at least three affected children. Linkage analysis revealed a region on chromosome 18 with a significant LOD score of 4.3. In this area, two homozygous nonconserved missense mutations in immediate early response 3 interacting protein 1 (IER3IP1) were found in patients from both families. IER3IP1 is highly expressed in the fetal brain cortex and fetal pancreas and is thought to be involved in endoplasmic reticulum stress response. We reported one of these families previously in a paper on Wolcott-Rallison syndrome (WRS). WRS is characterized by increased apoptotic cell death as part of an uncontrolled unfolded protein response. Increased apoptosis has been shown to be a cause of microcephaly in animal models. An autopsy specimen from one patient showed increased apoptosis in the cerebral cortex and pancreas beta cells, implicating premature cell death as the pathogenetic mechanism. Both patient fibroblasts and control fibroblasts treated with siRNA specific for IER3IP1 showed an increased susceptibility to apoptotic cell death under stress conditions in comparison to controls. This directly implicates IER3IP1 in the regulation of cell survival. Identification of IER3IP1 mutations sheds light on the mechanisms of brain development and on the pathogenesis of infantile epilepsy and early-onset permanent diabetes.Facultad de Ciencias Médica

The ALERT-ES Project for earthquakes in Cape San Vicente region

The main goal of the ALERT-ES project ("Sistema de Alerta Sismica Temprana: Aplicacion al Sur de España" ) is to study the feasibility of an Earthquake Early Warning System (EEWS) for the potentially damaging earthquakes that occur in the zone Cape S. Vicente-Gulf of Cadiz (S. Spain). This area is characterized by the occurrence of large and damaging earthquakes such as the 1755 Lisbon (Imax=X) or 1969 S. Vicente Cape (Ms=8,1) events. Most earthquakes in this area have their epicenters offshore at epicentral distances between 150 and 250 kms off the coast line, so a feasibility study is needed before an EEWS system implementation. The project has two different parts: the development of algorithms for the rapid estimation of the magnitude for South Spain earthquakes from the very beginning of P-waves and the development of the corresponding new software modules and their implementation in the EarthWorm and SeisComP systems. A pilot experience will be carried out during the project, using observations from coastal stations and OBS. Broadband records from a selection of 19 earthquakes (M≥4.0) occurred in the period 2006 to 2010 in Cape S. Vicente and Gulf of Cadiz have been used for an off line testing of PRESTO methodology developed at Naples University (Italy). Preliminary results show that for a Mw 6.1 shock with epicenter 200 km SW of Cape of S. Vicente the blind area has a radius of 227 km, providing with a lead-time of 28s in Huelva, 36s in Cadiz and 47s in Seville

Boussignac continuous positive airway pressure for the management of acute cardiogenic pulmonary edema: prospective study with a retrospective control group

<p>Abstract</p> <p>Background</p> <p>Continuous positive airway pressure (CPAP) treatment for acute cardiogenic pulmonary edema can have important benefits in acute cardiac care. However, coronary care units are usually not equipped and their personnel not adequately trained for applying CPAP with mechanical ventilators. Therefore we investigated in the coronary care unit setting the feasibility and outcome of the simple Boussignac mask-CPAP (BCPAP) system that does not need a mechanical ventilator.</p> <p>Methods</p> <p>BCPAP was introduced in a coronary care unit where staff had no CPAP experience. All consecutive patients transported to our hospital with acute cardiogenic pulmonary edema, a respiratory rate > 25 breaths/min and a peripheral arterial oxygen saturation of < 95% while receiving oxygen, were included in a prospective BCPAP group that was compared with a historical control group that received conventional treatment with oxygen alone.</p> <p>Results</p> <p>During the 2-year prospective BCPAP study period 108 patients were admitted with acute cardiogenic pulmonary edema. Eighty-four of these patients (78%) were treated at the coronary care unit of which 66 (61%) were treated with BCPAP. During the control period 66 patients were admitted over a 1-year period of whom 31 (47%) needed respiratory support in the intensive care unit. BCPAP treatment was associated with a reduced hospital length of stay and fewer transfers to the intensive care unit for intubation and mechanical ventilation. Overall estimated savings of approximately € 3,800 per patient were achieved with the BCPAP strategy compared to conventional treatment.</p> <p>Conclusion</p> <p>At the coronary care unit, BCPAP was feasible, medically effective, and cost-effective in the treatment of acute cardiogenic pulmonary edema. Endpoints included mortality, coronary care unit and hospital length of stay, need of ventilatory support, and cost (savings).</p

Microcephaly with simplified gyration, epilepsy, and infantile diabetes linked to inappropriate apoptosis of neural progenitors

We describe a syndrome of primary microcephaly with simplified gyral pattern in combination with severe infantile epileptic encephalopathy and early-onset permanent diabetes in two unrelated consanguineous families with at least three affected children. Linkage analysis revealed a region on chromosome 18 with a significant LOD score of 4.3. In this area, two homozygous nonconserved missense mutations in immediate early response 3 interacting protein 1 (IER3IP1) were found in patients from both families. IER3IP1 is highly expressed in the fetal brain cortex and fetal pancreas and is thought to be involved in endoplasmic reticulum stress response. We reported one of these families previously in a paper on Wolcott-Rallison syndrome (WRS). WRS is characterized by increased apoptotic cell death as part of an uncontrolled unfolded protein response. Increased apoptosis has been shown to be a cause of microcephaly in animal models. An autopsy specimen from one patient showed increased apoptosis in the cerebral cortex and pancreas beta cells, implicating premature cell death as the pathogenetic mechanism. Both patient fibroblasts and control fibroblasts treated with siRNA specific for IER3IP1 showed an increased susceptibility to apoptotic cell death under stress conditions in comparison to controls. This directly implicates IER3IP1 in the regulation of cell survival. Identification of IER3IP1 mutations sheds light on the mechanisms of brain development and on the pathogenesis of infantile epilepsy and early-onset permanent diabetes.Facultad de Ciencias Médica

Genotype–Phenotype Correlation in DFNB8/10 Families with TMPRSS3 Mutations

In the present study, genotype–phenotype correlations in eight Dutch DFNB8/10 families with compound heterozygous mutations in TMPRSS3 were addressed. We compared the phenotypes of the families by focusing on the mutation data. The compound heterozygous variants in the TMPRSS3 gene in the present families included one novel variant, p.Val199Met, and four previously described pathogenic variants, p.Ala306Thr, p.Thr70fs, p.Ala138Glu, and p.Cys107Xfs. In addition, the p.Ala426Thr variant, which had previously been reported as a possible polymorphism, was found in one family. All affected family members reported progressive bilateral hearing impairment, with variable onset ages and progression rates. In general, the hearing impairment affected the high frequencies first, and sooner or later, depending on the mutation, the low frequencies started to deteriorate, which eventually resulted in a flat audiogram configuration. The ski-slope audiogram configuration is suggestive for the involvement of TMPRSS3. Our data suggest that not only the protein truncating mutation p.T70fs has a severe effect but also the amino acid substitutions p.Ala306Thr and p.Val199Met. A combination of two of these three mutations causes prelingual profound hearing impairment. However, in combination with the p.Ala426Thr or p.Ala138Glu mutations, a milder phenotype with postlingual onset of the hearing impairment is seen. Therefore, the latter mutations are likely to be less detrimental for protein function. Further studies are needed to distinguish possible phenotypic differences between different TMPRSS3 mutations. Evaluation of performance of patients with a cochlear implant indicated that this is a good treatment option for patients with TMPRSS3 mutations as satisfactory speech reception was reached after implantation

Inherited variants in CHD3 show variable expressivity in Snijders Blok-Campeau syndrome

Purpose: Common diagnostic next-generation sequencing strategies are not optimized to identify inherited variants in genes associated with dominant neurodevelopmental disorders as causal when the transmitting parent is clinically unaffected, leaving a significant number of cases with neurodevelopmental disorders undiagnosed. Methods: We characterized 21 families with inherited heterozygous missense or protein-truncating variants in CHD3, a gene in which de novo variants cause Snijders Blok-Campeau syndrome. Results: Computational facial and Human Phenotype Ontology–based comparisons showed that the phenotype of probands with inherited CHD3 variants overlaps with the phenotype previously associated with de novo CHD3 variants, whereas heterozygote parents are mildly or not affected, suggesting variable expressivity. In addition, similarly reduced expression levels of CHD3 protein in cells of an affected proband and of healthy family members with a CHD3 protein-truncating variant suggested that compensation of expression from the wild-type allele is unlikely to be an underlying mechanism. Notably, most inherited CHD3 variants were maternally transmitted. Conclusion: Our results point to a significant role of inherited variation in Snijders Blok-Campeau syndrome, a finding that is critical for correct variant interpretation and genetic counseling and warrants further investigation toward understanding the broader contributions of such variation to the landscape of human disease