Search

Das Guillain-Barré-Syndrom (GBS; Synonyma: Polyneuritis, Polyradikulitis, Akute entzündliche demyelinisierende Polyradikuloneuropathie, Polyradikuloneuritis) ist eine akute, entzündliche, demyelinisierende Erkrankung des peripheren Nervensystems. Die Erkrankung präsentiert sich klinisch mit symmetrischen Lähmungen, Areflexie und ausgeprägten vegetativen Störungen. Respiratorisches Versagen und kardiovaskuläre Instabilität sind die Hauptgründe für die Übernahme eines Patienten mit GBS auf die Intensivstation. Der vorliegende Bericht schildert den ungewöhnlichen schweren Verlauf einer Polyradikuloneuritis bei einem 10 Monate alten Säugling und diskutiert RS-Viren als mögliche Auslöser. Bei einem ARDS im Kindesalter könnte eine Stickoxidbeatmung (NO) eine lebensrettende Option darstellen.

Glacier melt is an essential source of freshwater for the arid regions surrounding the Tian Shan. However, the knowledge about glacier volume and mass changes over the last decades is limited. In the present study, glacier area, glacier dynamics and mass changes are investigated for the period ~1975–2007 for Southern Inylchek Glacier (SIG) and Northern Inylchek Glacier (NIG), the largest glacier system in Central Tian Shan separated by the regularly draining Lake Merzbacher. The area of NIG increased by 2.0 ± 0.1 km2 (~1.3%) in the period ~1975–2007. In contrast, SIG has shrunk continuously in all investigated periods since ~1975. Velocities of SIG in the central part of the ablation region reached ~100–120 m a−1 in 2002/2003, which was slightly higher than the average velocity in 2010/2011. The central part of SIG flows mainly towards Lake Merzbacher rather than towards its terminus. The measured velocities at the distal part of the terminus downstream of Lake Merzbacher were below the uncertainty, indicating very low flow with even stagnant parts. Geodetic glacier mass balances have been calculated using multi-temporal digital elevation models from KH-9 Hexagon (representing the year 1975), SRTM3 (1999), ALOS PRISM (2006) and SPOT-5 high-resolution geometrical (HRG) data (2007). In general, a continuous mass loss for both SIG and NIG could be observed between ~1975 and 2007. SIG lost mass at a rate of 0.43 ± 0.10 m w.e. a−1 and NIG at a rate of 0.25 ± 0.10 m w.e. a−1 within the period ~1975–1999. For the period 1999–2007, the highest mass loss of 0.57 ± 0.46 m w.e. a−1 was found for NIG, whilst SIG showed a potential moderate mass loss of 0.28 ± 0.46 m w.e. a−1. Both glaciers showed a small retreat during this period. Between ~1975 and 1999, we identified a thickening at the front of NIG with a maximum surface elevation increase of about 150 m as a consequence of a surge event. In contrast significant thinning (>0.5 m a−1) and comparatively high velocities close to the dam of Lake Merzbacher were observed for SIG, indicating that Lake Merzbacher enhances glacier mass loss.

A 41 year old woman presented with dyspnoea at rest and swollen legs in the emergency room of our centre. She reported a history of slowly progressing dyspnoea and oedema in the legs. Physical examination showed signs of biventricular congestive heart failure and dysmorphia of the face. Routine laboratory examination revealed elevated CK levels without significant elevations of the CK-MB isoform. ECG showed complete left bundle branch block and first degree atrioventricular block. Echocardiography and angiography showed markedly reduced left ventricular systolic function, the ejection fraction was 25%. Coronary angiography excluded CAD and there was no evidence for congenital or valvular heart disease. The patient also reported a history of a serious complication during emergency general anaesthesia and cataracts of both eyes. Because of the clinical and chemical findings, the history of cataracts and complications during general anaesthesia, a systemic congenital disease of the muscular tissue was suspected. Molecular studies revealed a trinucleotide amplification at the myotonic dystrophy locus 19q 13.3, so the diagnosis myotonic dystrophy Curschmann-Steinert was established. The sixteen year old son of the patient suffered from an at this time unknown disease with retardation, muscular weakness and myotonia of the face. The diagnosis myotonic dystrophy was evident because of the clinical signs and the family history.

Im Jahr 1953 fuhrte Lichtenstein den Begriff der Histiocytosis X ein [3]. Immunhistologische Untersuchungen ergaben die uberwiegend monoklonale Proliferation von CD-1-positiven Histiozyten (Langerhans-Zellen), sodass 1987 die Erkrankung dieses Formenkreises unter dem Begriff der Langerhans-Zell-Histiozytose (LCH) zusammengefasst wurde [6]. Die LCH stellt ein seltenes Krankheitsbild dar. Geschatzt wird eine Inzidenz von ca. 0,5 pro 100.000 Einwohner [1]. Die genaue Inzidenz und Pravalenz der Erkrankung sind unbekannt. Die primar pulmonale Langerhans-Zell-Histiozytose ist eine Erkrankung im Erwachsenenalter. Vorwiegend im Kleinkindesalter tritt die rasch progressive, disseminierte Form (Abt-Lettlerer-Siwe-Erkrankung) auf. Der M. Hand-Schuller-Christian manifestiert sich uberwiegend im Jugendalter. Der beschriebene Patient zeigt die progressive, disseminierte Form dieser Erkrankung.

Long-term monitoring of water resources and climate parameters at the scale of river basins requires networks of continuously operated in-situ stations. Since 2009, GFZ and CAIAG, in cooperation with the National Hydrometeorological Services (NHMS) of Central Asia, are establishing such a regional monitoring network in Kazakhstan, Kyrgyzstan, Tajikistan, Uzbekistan, and lately Afghanistan to collect observations of meteorological and hydrological parameters and to deliver them to the end-users for operational tasks and scientific studies. The newly developed and installed remotely operated multi-parameter stations (ROMPS) do not only monitor standard meteorological and hydrological parameters, but also deliver Global Navigation Satellite System (GNSS) data for atmospheric sounding as well as tectonic studies. Additionally, three stations integrate seismic sensors for earthquake monitoring. The observational data from the ROMPS is transmitted nominally in near-real time, but at least once a day to a centralized geo-database infrastructure for long-term storage and data redistribution. Users can access the data manually using a web-interface or automatically using SOS requests; in addition, data is planed to be distributed to the NHMS through standard communication and data exchange channels.

Long-term monitoring of water resources and climate parameters at the scale of river basins requires networks of continuously operated in-situ stations. Since 2009, GFZ and CAIAG, in cooperation with the National Hydrometeorological Services (NHMS), are establishing such a regional monitoring network in Central Asia (Kazakhstan, Kyrgyzstan, Tajikistan, Uzbekistan, and Afghanistan) which is collecting observations of meteorological and hydrological parameters and delivering them to the end-users. The network design focuses mainly on the higher elevations where the recent decline of monitoring stations and networks established in Soviet times was strongest, and the resulting observational gap hinders research on climate and hydrological change as well as operational tasks in water management such as the seasonal runoff forecast. The newly developed and installed Remotely Operated Multi-Parameter Stations (ROMPS) do not only monitor standard meteorological and hydrological parameters, but also deliver GPS data for atmospheric sounding as well as tectonic studies. The observational data from the ROMPS is transmitted at least once a day to a centralized geo-database infrastructure for long-term storage and data redistribution. Users can access the data manually using a web-interface or automatically using SOS requests; in addition, data is distributed to the NHMS through standard communication and data exchange channels.

Detailed analysis of stored electrograms is essential for the interpretation of arrhythmias, programming changes, and optimization of the medical therapy in patients with implanted pacemakers and defibrillators. The physician who cares for patients with implantable electrical devices has to be able to understand the detection and treatment algorithms of those devices. Biotronik pacemakers of newer generations are capable of storing intracardiac electrograms. Earlier devices store up to 12 electrograms of 10 s duration after certain trigger events, like atrial tachycardia or high ventricular rates. Cardiac resynchronization systems can store electrograms after patient activation with magnets in addition to the above mentioned trigger-activated electrograms. Defibrillators store intracardiac electrograms during tachycardia episodes with near-field and far-field electrograms of the right ventricular lead in addition to the markers in single and dual chamber defibrillators (in addition to an atrial electrogram) and near field electrograms of the atrial, the right, and the left ventricular electrode in addition to the markers in resynchronization systems. Each channel has a maximum storing capacity of 32 min. If there are more episodes than storing capacity, electrograms of older episodes will be overwritten, but if the newer episodes are all classified as supraventricular, the last two ventricular episodes (VT or VF) will remain in the episode memory. This article describes stored electrograms, detection, and treatment algorithms of implantable cardiac devices manufactured by Biotronik.

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia and its prevalence is increasing with age. With aging of the population treatment of atrial fibrillation especially in elderly population is a growing task for all medical staff working with elderly patients. Treatment of atrial fibrillation especially in elderly patients has to focus on prevention of thromboembolism as well as symptom relief with rate or rhythm control. This review article will focus on medical and non-pharmacological treatment options for the treatment of atrial fibrillation in elderly patients.

Fault zones are the locations where motion of tectonic plates, often associated with earthquakes, is accommodated. Despite a rapid increase in the understanding of faults in the last decades, our knowledge of their geometry, petrophysical properties, and controlling processes remains incomplete. The central questions addressed here in our study of the Dead Sea Transform (DST) in the Middle East are as follows: (1) What are the structure and kinematics of a large fault zone? (2) What controls its structure and kinematics? (3) How does the DST compare to other plate boundary fault zones? The DST has accommodated a total of 105 km of left-lateral transform motion between the African and Arabian plates since early Miocene (similar to 20 Ma). The DST segment between the Dead Sea and the Red Sea, called the Arava/Araba Fault (AF), is studied here using a multidisciplinary and multiscale approach from the mu m to the plate tectonic scale. We observe that under the DST a narrow, subvertical zone cuts through crust and lithosphere. First, from west to east the crustal thickness increases smoothly from 26 to 39 km, and a subhorizontal lower crustal reflector is detected east of the AF. Second, several faults exist in the upper crust in a 40 km wide zone centered on the AF, but none have kilometer-size zones of decreased seismic velocities or zones of high electrical conductivities in the upper crust expected for large damage zones. Third, the AF is the main branch of the DST system, even though it has accommodated only a part (up to 60 km) of the overall 105 km of sinistral plate motion. Fourth, the AF acts as a barrier to fluids to a depth of 4 km, and the lithology changes abruptly across it. Fifth, in the top few hundred meters of the AF a locally transpressional regime is observed in a 100-300 m wide zone of deformed and displaced material, bordered by subparallel faults forming a positive flower structure. Other segments of the AF have a transtensional character with small pull-aparts along them. The damage zones of the individual faults are only 5-20 m wide at this depth range. Sixth, two areas on the AF show mesoscale to microscale faulting and veining in limestone sequences with faulting depths between 2 and 5 km. Seventh, fluids in the AF are carried downward into the fault zone. Only a minor fraction of fluids is derived from ascending hydrothermal fluids. However, we found that on the kilometer scale the AF does not act as an important fluid conduit. Most of these findings are corroborated using thermomechanical modeling where shear deformation in the upper crust is localized in one or two major faults; at larger depth, shear deformation occurs in a 20-40 km wide zone with a mechanically weak decoupling zone extending subvertically through the entire lithosphere.

Guillain Barré syndrome (GBS; Synonyma: Polyneuritis, Polyradikulitis) is an acute, inflammatory, demyelinating disease of the peripheral nerve system. Clinical hallmarks are symmetric muscle paralysis, areflexia and pronounced autonomic disturbances. Respiratory failure and cardiovascular instability are the main reasons for intensive care support in patients with GBS. We present the process of illness of an 10 month old baby with GBS. The report discusses RSV as possible triggers. For children with ARDS, a nitric oxide ventilation could represent a lifesaving option.

Tracheal agenesis (TA), aplasia or total atresia of the trachea are congenital anomalies which are still incompatible with life. Despite the many attempts of different interventions, there are yet no promising, long-term methods of treatment. Only with sufficient proportion of the proximal or distal trachea available, it is possible to place a tracheostomy, which also opens up new vistas of life for the affected child. In most cases the seldom deformation, trachealagenesis, does not get recognised before the child is born. It may therefore be the immediate diagnosis postnatal that is decisive over the final prognosis of the child. The prepartal suspicion of a duodenal stenosis, an aphonic newborn as well as the frustrane attempts of intubation are possible guidelines of TA. In independence of peripartal and anamnestical factors, individual disciplinary decisions are necessary for further treatments. After the cancellation of intensive care the premature infant of the case report died as consequence of postnatal diagnosed tracheal aplasia. Under circumstances, medical treatments such as the ex utero intrapartum procedure (Exit), the temporary method of extracorporal membrane oxygenation (ECMO) or the use of cartilage tissue for the plastic trachea reconstruction can provide advanced medical opportunities.

The implantation of transvenous devices in patients who underwent tricuspid valve replacement represents problems, especially if an epicardial position is not available. The implantation of a "pace-sense" lead via the coronary sinus is a safe and feasible procedure. For experienced surgeons in implantation of biventricular devices, the implantation of leads via the coronary sinus is a routine procedure. Bipolar leads are essential for the correct sensing and pacing of the implantable cardioverter-defibrillator (ICD). In patients who underwent tricuspid valve replacement who have the indication of an ICD implantation postoperatively, the combination of a shock electrode placed in the superior vena cava, a subcutaneous array positioned on the left posterior close to the spine and an active can, placed subpectorally in the left infraclavicular region, is an alternative solution.

The northeast Ethiopian plateau is composed of a low-grade metamorphic complex which includes the Tsaliet Metavolcanics overlain by the NE-trending upright folded metasediments of the Tambien Group including a thick stromatolitic carbonate sequence. High-grade metasediments and orthogneisses structurally underlie this unit in the Red Sea Lowlands of east Eritrea. In east Eritrea, the low-grade Bizen Domain metasediments and metavolcanics structurally overlie the Ghedem Domain high-grade rocks with a subhorizontal moderately W-dipping transition zone of tectonic origin. Field studies augmented by remote sensing analysis of Modular Optoelectronic Multispectral Stereo Scanner on Priroda (MOMS-2P) images are used to outline the structure, stratigraphy and geomorphology and the continuation of the Negash Synclinorium with adjacent structures up to the Bizen Domain in east Eritrea. The protolith age of the low- and high-grade rocks is around 850 and 800 Ma, respectively. Peak metamorphic conditions in the high-grade metasediments reached 700 ◦ C and up to 12 kbar (depth of ∼40 km) at ∼650 Ma. This indicates that an elevated mountain belt with a very thick crust existed in Eritrea during the evolution of the East African Orogen (EAO). Rapid exhumation of the high-grade metamorphic rocks occurred between 640 and 545 Ma involving extension along a tectonic transition zone. The transition zone is a ductile detachment overprinted by brittle low-angle faulting. Although partly pertaining to a Tertiary deformation related to the opening of the Red Sea, we suggest that this low-angle zone was a detachment surface in the Neoproterozoic that accommodated extension during orogenic collapse to restore normal crustal thickness which was thickened due to collision between East and West Gondwana. The sedimentary rocks of the Tambien Group in north Ethiopia and the Bizen Domain in east Eritrea (stromatolitic limestone, stromatolitic dolostone, polymict conglomerate-diamictites), their stratigraphy and age of deposition suggest association with “Snowball Earth”. Light δ 13 C( −6.74 and −6.88‰) were measured for the stromatolitic dolostone and algal mat of the Bizen Domain which are characteristic for cap-carbonates associated with Snowball Earth. © 2003 Elsevier Science B.V. All rights reserved.

A 41 year old woman presented with dyspnoea at rest and swollen legs in the emergency room of our centre. She reported a history of slowly progressing dyspnoea and oedema in the legs. Physical examination showed signs of biventricular congestive heart failure and dysmorphia of the face. Routine laboratory examination revealed elevated CK levels without significant elevations of the CK-MB isoform. ECG showed complete left bundle branch block and first degree atrioventricular block. Echocardiography and angiography showed markedly reduced left ventricular systolic function, the ejection fraction was 25%. Coronary angiography excluded CAD and there was no evidence for congenital or valvular heart disease. The patient also reported a history of a serious complication during emergency general anaesthesia and cataracts of both eyes. Because of the clinical and chemical findings, the history of cataracts and complications during general anaesthesia, a systemic congenital disease of the muscular tissue was suspected. Molecular studies revealed a trinucleotide amplification at the myotonic dystrophy locus 19q 13.3, so the diagnosis myotonic dystrophy Curschmann-Steinert was established. The sixteen year old son of the patient suffered from an at this time unknown disease with retardation, muscular weakness and myotonia of the face. The diagnosis myotonic dystrophy was evident because of the clinical signs and the family history.

spectively), (p

Es wird der verhaltnismasig seltene, aber wegen der Operationsmoglichkeit wichtige Befund eines arteriovenosen Aneurysma der Arteria pulmonalis bei einer Teleangiektasia haemorrhagica hereditaria Osler dargestellt. Die Diagnose konnte durch Feststellung eines systolischen Gerauschs in der rechten hinteren Axillarlinie, herabgesetzte arterielle Sanerstoffsattigung und den charakteristischen Rontgenbefund von uber Jahre konstanten Rundschatten mit breiter bandformiger Verbindung zum Hilus geklart werden. Wiederholte Magendarmblutungen sind mit Wahrscheinlichkeit ebenfalls auf Teleangiektasien zuruckzufuhren, doch last sich dies nachtraglich nicht mehr mit Sicherheit sagen. Eine weitere Anomalie der Lungengefase in Form einer allgemeinen Ektasie der Art. pulmonalis des rechten Unterlappens wird unter Hinzuziehung des Schrifttums uber derartige Varietaten erortert. The comparative rare, but because of the surgical possibility important finding of an arteriovenous aneurysm of the pulmonary trunk in a case of a teleangiectasia haemorrhagica hereditaria Osler is demonstrated. The diagnosis could be cleared by verification of a systolic murmur in the right posterior midaxillary line, by reduced arterial oxygen saturation and by the characteristic roentgenfinding of a over years constant round shadow with broad bandlike connection to the hilum. Repeated gastro-intestinal haemorrhage also seems to be due to telangiectasia, although this can later on not be relied upon. A further anomaly of the pulmonary vessels in form of a general ectasia of the pulmonary trunk of the right lower lobe and similar varieties in the literature are discussed. Description d'un cas d'anevrysme arterio-veineux de l'artere pulmonaire dans un cas de maladie d'Osler- Rendu (teleangiectasie hemorragique hereditaire), localisation rare mais importante parce que susceptible d'etre operee. Un souffle systolique sur la ligne axillaire posterieure droite, une diminution de la saturation en oxygene du sang arteriel et une opacite ronde inchangee pendant une observation de plusieurs annees, et en rapport avec le hile ont permis de poser le diagnostic. Des hemorragies gastro-intestinales a repetition sont vraisemblablement dues a la presence de teleangiectasies. Le cas presente egalement une ectasie des branches de l'artere pulmonaire droite dans le lobe inferieur, anomalie decrite deja par d'autres auteurs. Se describe un caso de aneurisma arterio-venoso de la arteria pulmonar en un cuadro de telangiectasia hemorragica hereditaria de Osler, cuadro relativamente raro pero importante de conocer si se tiene en cuenta la posibilidad de su tratamiento quirurgico. El diagnostico pudo ser aclarado por la comprobacion de la presencia de un soplo sistolico a nivel de la linca axilar dereeha posterior, por la disminuida saturacion arterial del oxigeno y la presencia, por anos, en la radiografia, de una imagen redondeada unida al hilio por medio de gruesos tractos. Las repetidas hemorragias gastro-intestinales pueden ser atribuidas probablemente tambien a telangiectasias, si bien es dificil afirmarlo a ulteriori. Otra anomalia, que se senala como variante, esta representada por una ectasia generalizada de la arteria pulmonar del lobulo inferior derecho, modalidad que se comenta a base de la bibliografia correspondiente.