Search

We propose a global geomagnetic field model for the last fourteen thousand years, based on thermoremanent records. We call the model ArchKalmag14k. ArchKalmag14k is constructed by modifying recently proposed algorithms, based on space-time correlations. Due to the amount of data and complexity of the model, the full Bayesian posterior is numerically intractable. To tackle this, we sequentialize the inversion by implementing a Kalman-filter with a fixed time step. Every step consists of a prediction, based on a degree dependent temporal covariance, and a correction via Gaussian process regression. Dating errors are treated via a noisy input formulation. Cross-correlations are re-introduced by a smoothing algorithm and model parameters are inferred from the data. Due to the specific statistical nature of the proposed algorithms, the model comes with space and time dependent uncertainty estimates. The new model ArchKalmag14k shows less variation in the large scale degrees than comparable models. Local predictions represent the underlying data and agree with comparable models, if the location is sampled well. Uncertainties are bigger for earlier times and in regions of sparse data coverage. We also use ArchKalmag14k to analyze the appearance and evolution of the South Atlantic anomaly together with reverse flux patches at the core mantle boundary, considering the model uncertainties. While we find good agreement with earlier models for recent times, our model suggests a different evolution of intensity minima prior to 1650 CE. In general, our results suggest that prior to 6000 BCE the data is not sufficient to support global models.

International audience

Intestinal Dysganglionoses (IDs) represent a heterogeneous group of Enteric Nervous System anomalies including Hirschsprung's disease (HD), Intestinal Neuronal Dysplasia (IND), Internal Anal Sphincter Neurogenic Achalasia (IASNA) and Hypoganglionosis. At present HD is the only recognised clinico-pathological entity, whereas the others are not yet worldwide accepted and diagnosed. This report describes the areas of agreement and disagreement regarding definition, diagnosis, and management of IDs as discussed at the workshop of the fourth International Meeting on "Hirschsprung's disease and related neurochristopathies." The gold standards in the preoperative diagnosis of IDs are described, enlighting the importance of rectal suction biopsy in the diagnostic workup. The most important diagnostic features of HD are the combination of hypertrophic nerve trunks and aganglionosis in adequate specimens. Acetylcholinesterase staining is the best diagnostic technique to demonstrate hypertrophic nerve trunks in lamina propia mucosae, but many pathologist from different centers still use H&E staining effectively. Moreover, the importance of an adequate intraoperative pathological evaluation of the extent of IDs to avoid postoperative complications is stressed. Although it is not clear whether IND is a separate entity or some sort of secondary acquired condition, it is concluded that both IND and IASNA do exist. Other interesting conclusions are provided as well as detailed results of the discussion. Further investigation is needed to resolve the many controversies concerning IDs. The fourth International Conference in Sestri Levante stimulated discussion regarding these entities and led to the International guidelines to serve the best interest of our patients.

But: Le traitement des malformations intestinales neuronales longues posent au chirurgien pediatre des problemes diagnostiques, therapeutiques et de suivi post-operatoire. La seule presence de cellules ganglionnaires est insuffisante pour decider de la longueur de la resection et n'exclue pas une hypoganglionose et/ou une dysganglionose disseminee. Les techniques de creation de poches aussi bien que les techniques usuelles de resections selon Rehbein, Soave et Duhamel sont discutees. Les etudes avec un grand nombre de patients sont rares; nous presentons dans ce travail nos propres resultats. Methodes: 48 patients avec une malformation neuronale intestinale longue ont ete traites dans notre hopital de 1990 a 2000. Un total de 35 patients ont ete vus 1,5 a 6 ans apres le traitement chirurgical definitif. Dans tous les cas, une resection anterieure de Rehbein etait realisee. Resultats: Nos resultats montrent que le traitement chirurgical avec la technique de Rehbein donne de bons resultats en ce qui concerne le risque de complications aussi bien que l'evolution post-operatoire, alors qu'un segment aganglionnaire de 4 cm demeure in situ. Nous trouvons de meilleurs resultats apres ascendo-rectostomie (n=22) compare a l'ileo-rectostomie (n=11). Suivant les premieres publications de ce groupe (13), l'histologie de la marge proximale est decisive pour le pronostic. L'hypoganglionose ou l'aganglionose doit conduire a une resection alors que de courts segments intestiaux neurodysplasiques du colon ou de l'ileon terminal peuvent rester en place. Cependant, la presence d'un segment aganglionaire rectal distal et la diminution du peristaltisme de l'intestin pre-anastomotique doivent etre pris en compte. Plusieurs investigations sont necessaires pour savoir si la combinaison de l'abaissement endo-rectal selon Soave avec un intestin proximal dysplasique peut donner de meilleurs resultats ou augmenter la frequence de l'enterocolite post-operatoire et de l'incontinence. Un diagnostic precis de l'innervation de la paroi intestinale est decisive pour un traitement optimal et en considerant nos resultats, il est essentiel que dans le cas d'un nouveau-ne hospitalise avec une sub-obstruction colique et suspicion d'une malformation intestinale neuronale, des biopsie de toute la paroi du colon distal et proximal soient faites en meme temps que l'enterostomie. Generalement, l'ileostomie est realisee chez les patients chez lesquels on suspecte une malformation intestinale a long segment. Avant la resection definitive, des biopsies doivent etre faites au niveau des stomies.

What is the training in pediatric surgery like, how is pediatric surgery defined in other countries and what are the possibilities of quality control for training in pediatric surgery? The results of an inquiry together with information about training in pediatric surgery in 24 countries are summarised to show the different possibilities of organising training in pediatric surgery inside and outside Europe. The number of trainees in pediatric surgery (ranging from 0 to 339 trainees per country), the density of pediatric surgeons (ranging from 424 to 35 714 live births/year per pediatric surgeon) and the number of Centres of Pediatric Surgery (ranging from 4167 to 65,000 live births or 450,000 to 5,300,000 inhabitants per Centre) varies a lot in the different countries. Countries with a higher density of pediatric surgeons often also have a higher number of trainees irrespective of the birth rate and the number of inhabitants within the country, indicating a good infrastructure for pediatric surgery. In 87.5 % of the countries pediatric surgery is recognised as a specialty. The mean duration of training is about 6 years and 3 months, excluding the countries where it is necessary to become a fully accredited general surgeon before doing pediatric surgery. The mean duration of stay in general surgery is 2 years and 10 months. In one third of the countries it is obligatory to spend some time (3 to 12 months) in pediatrics. An elective or a compulsory period of time (1 - 6 months) in other specialties such as orthopedic surgery, plastic and reconstructive surgery, thoracic surgery and urology exists in 41.7 % of the nations. The most common subspecialties within pediatric surgery practised during the training by the resident are abdominal surgery, thoracic surgery, oncological surgery, head and neck surgery and urology. Possible means of control to guarantee a high quality of training include a defined time of stay in each subspecialty, the number of operations done by the trainee or the number of cases treated by the resident in the respective subspecialty and the distinction of different levels of surgery done by the trainee during his residence. In many cases the type of control is not specified.

The theoretically studied impact of rapid rotation on stellar evolution needs to be confronted with the results of high resolution spectroscopy-velocimetry observations. A weak surface magnetic field had recently been detected in the A0 prototype star Vega, potentially leading to a (yet undetected) structured surface. The goal of this article is to present a thorough analysis of the line profile variations and associated estimators in the early-type standard star Vega (A0) in order reveal potential activity tracers, exoplanet companions and stellar oscillations. Vega was monitored in high-resolution spectroscopy with the velocimeter Sophie/OHP. A total of 2588 high S/N spectra was obtained during 5 nights (August 2012) at R = 75000 and covering the visible domain. For each reduced spectrum, Least Square Deconvolved (LSD) equivalent photospheric profiles were calculated with a Teff = 9500 and logg = 4.0 spectral line mask. Several methods were applied to study the dynamic behavior of the profile variations (evolution of radial velocity, bisectors, vspan, 2D profiles, amongst others). We present the discovery of a starspotted stellar surface in an A-type standard star with faint spot amplitudes Delta F/Fc ~5 10^{-4}. A rotational modulation of spectral lines with a period of rotation P = 0.68 d has clearly been exhibited, confirming the results of previous spectropolarimetric studies. Either a very thin convective layer can be responsible for magnetic field generation at small amplitudes, or a new mechanism has to be invoked in order to explain the existence of activity tracing starspots. This first strong evidence that standard A-type stars can show surface structures opens a new field of research and asks the question about a potential link with the recently discovered weak magnetic field discoveries in this category of stars., Comment: accepted for publication by Astronomy & Astrophysics (23rd of March 2015)

The magnetosphere-ionosphere-thermosphere (MIT) dynamic system significantly depends on the highly variable solar wind conditions, in particular, on changes of the strength and orientation of the interplanetary magnetic field (IMF). The solar wind and IMF interactions with the magnetosphere drive the MIT system via the magnetospheric field-aligned currents (FACs). The global modeling helps us to understand the physical background of this complex system. With the present study, we test the recently developed high-resolution empirical model of field-aligned currents MFACE (a high-resolution Model of Field-Aligned Currents through Empirical orthogonal functions analysis). These FAC distributions were used as input of the time-dependent, fully self-consistent global Upper Atmosphere Model (UAM) for different seasons and various solar wind and IMF conditions. The modeling results for neutral mass density and thermospheric wind are directly compared with the CHAMP satellite measurements. In addition, we perform comparisons with the global empirical models: the thermospheric wind model (HWM07) and the atmosphere density model (Naval Research Laboratory Mass Spectrometer and Incoherent Scatter Extended 2000). The theoretical model shows a good agreement with the satellite observations and an improved behavior compared with the empirical models at high latitudes. Using the MFACE model as input parameter of the UAM model, we obtain a realistic distribution of the upper atmosphere parameters for the Northern and Southern Hemispheres during stable IMF orientation as well as during dynamic situations. This variant of the UAM can therefore be used for modeling the MIT system and space weather predictions.

The upper atmosphere significantly depends on the highly variable solar wind conditions, in particular on changes of the strength and orientation of the Interplanetary Magnetic Field (IMF). The distribution of magnetospheric Field Aligned Currents (FAC) is the most important external driver for the complex system of the Earth’s environment, in particular the high-latitude ionosphere and thermosphere. The FAC distribution is closely related to the magnetospheric electric field distribution, which maps into the ionosphere. The ion drag together with energy deposition via Joule heating and particle precipitations drive the neutral upper atmosphere variations. All of those processes are parts of the Magnetosphere – Ionosphere – Thermosphere (MIT) dynamic system. The aim of this study is to test the recently developed empirical model of the Field-Aligned Currents MFACE. For this purpose the current distributions calculated with MFACE model were used as input of the time-dependent, fully self-consistent global Upper Atmosphere Model (UAM) for different seasons and various solar wind and IMF conditions. To quantify the quality of the theoretical model prediction the simulation results were compared with satellite observations and other models. The direct comparison with the CHAMP accelerometer measurements was done for the thermospheric wind data and the neutral mass density. A statistical analysis was performed for differences between the UAM, NRLMSISE-00, HWM07 models and the CHAMP data. The theoretical model shows a similar behavior as the empirical models. The new configuration of the UAM model reproduces realistic parameters and the M-I-T dynamics of the upper atmosphere for the Northern and Southern Hemispheres during stable IMF orientation as well as during dynamic situations. This variant of the UAM can be therefore used for space weather predictions.

The peristaltic movement of the gut is a function of the alternating contraction and relaxation of circular and longitudinal muscles. This movement is induced by a tendon-like connective-tissue net (TCTN) in the circular and longitudinal muscles, which are both rooted in a connective-tissue plexus layer (CTPL). In children with a therapy-resistant aperistaltic or hypoperistaltic syndrome who had normally-developed enteric innervation, a lack of the TCTN in the muscularis propria was observed. Over the last 2 years, 241 children with chronic constipation were investigated histopathologically; 46 children surgically treated by partial resection of the gut or diagnostically investigated by whole-mount biopsy. Fifteen children had a hypoperistalsis syndrome and 3 had an aperistalsis syndrome. All specimens were native and cut in a cryostat. Enteric innervation was examined by acetylcholinesterase and dehydrogenase reactions. The TCTN in the muscularis propria was stained with picric acid/sirius red. In the children with an aperistalsis syndrome, a complete lack of the TCTN in circular and longitudinal muscles was observed. A CTPL was not developed. The children with a hypoperistalsis syndrome had no CTPL, but had a partly-developed TCTN in the circular and longitudinal muscles, which gradually faded in the direction of the plexus layer. Independent of a well-developed enteric nervous system, a lack of the TCTN in longitudinal and circular muscles and a missing CTPL (aplastic desmosis) abolishes the coordinated peristaltic movement of the gut. An isolated lack of the CTPL in the myenteric plexus (hypoplastic desmosis) results in a hypoperistalsis syndrome. An anomaly of the TCTN in the muscularis propria disturbs gut-muscle mechanics, and is another cause of gut dysmotility.

The objective of our study was to determine the efficacy of ventriculostomy as the primary treatment for posthemorrhagic hydrocephalus in premature infants. Within a period of 4 years, 20 very low birthweight (VLBW) infants (birthweight median 1,135 g, range 650-1,470 g) were treated for progressive posthemorrhagic hydrocephalus (PHHC) by right parietal ventriculostomy (Salmon Rickham) at a mean age of 21 days. Serial tapping of the subcutaneous reservoir was performed for temporary drainage until conversion to a permanent ventriculoperitoneal (VP) shunt or spontaneous resolution of hydrocephalus. A total of 1,402 punctures (median 71/infant, range 13-168) was performed. The results showed that only 1/20 patients developed a cerebrospinal fluid (CSF) infection, accounting for a 5% patient-related and 0.07% procedure-related infection rate. Major complications such as skin defects, subdural hygroma, or CSF leaks occurred in three patients (15%). A permanent shunt was needed in 17 patients (85%). We concluded that, as an effective alternative to serial or lumbar puncture, there should be early implantation of ventriculostomy reservoirs for serial taps to control intracranial pressure in PHHC of VLBW infants until a permanent shunt can be placed because of the low incidence of infections and technical complications.

The authors present an overview of 101 patients operated for Hirschsprung's disease by Rehbein procedure. Special attention is directed to the histomorphological findings in the proximal segment of the resected bowel specimens. A strong link was confirmed between these specific features and postoperative bowel function. A histologically regular proximal bowel segment generally predicted good postoperative bowel function with a constipation rate of 10.5%. In patients with intestinal neuronal dysplasia (IND) of the proximal segment the overall clinical result remained unchanged, although the constipation rate rose to 17.8%. Postoperative bowel function was seriously affected in cases characterized by proximal segment hypoganglionosis with a constipation rate of 23.1% and an additional 7.7% with encopresis. Finally, the distinct group of children with aganglionosis of the proximal segment followed a complicated postoperative course with secondary bowel resections and recurrent episodes of enterocolitis. In addition, the authors state their general observation that histological findings become less important whenever a more extensive resection than left hemicolectomy is required. Discussing the results, guidelines are given to further patient treatment once the particular proximal segment histology is diagnosed.

Une nouvelle prothese de continence anale en polyurethane a ete testee en comparaison avec la prothese classique en PVA lors d'une etude croisee randomisee de 3 semaines chez 38 patients atteints d'incontinence partielle ou totale apres reparation d'une imperforation anale (âge 6-15 ans). Le controle de la defecation et les problemes en rapport avec la manipulation de la prothese ont ete enregistres avant l'etude, au moment du changement de prothese et apres l'etude. Resultats: Quinze des 38 patients n'ont pas termine le protocole. Parmi ces 15 patients, 6 avaient un diametre du canal anal trop faible pour la plus petite des protheses. Au cours de l'utilisation, la perception de remplissage et de besoin de defecation etait amelioree au cours de l'utilisation de la nouvelle prothese. La consistance des selles n'a pas ete modifiee chez 82% des patients. Le taux de constipation restait inchange (8/23 patients). Douze des 23 patients ayant complete le protocole ont acquis une proprete totale avec l'une ou l'autre prothese. Quinze patients (68%) utilisant le modele en polyurethane et 10 (45%) utilisant le modele classique PVA etaient confiant de ne pas se salir pendant son utilisation. Une insertion douloureuse, une sensation de pression a l'interieure du canal anal et des douleurs lors de l'extraction etaient rapportes pour les deux modeles, bien que moins frequemment avec la prothese en polyurethane. Conclusion: Les protheses anales de continence, quel que soit le modele, ont permis une proprete totale de quelques heures chez 66% de nos patients incontinents. Le modele en polyurethane (Conveen®, Coloplast) est prefere par les patients et permet plus de securite que le modele en PVA.

SUMMARY In this theoretical paper we show how to solve a time-dependent diiusion equation by means of a perturbation series. This technique is applied to the case of diiusion of a liquid through a time-dependent porous matrix. We compute to ¢rst order the phase and amplitude relations between the small deformation of the transporting matrix and the corresponding variation of the saturation at the surface. In particular we show that, for a large frequency range, there is a constant phase shift of n/2 between the matrix and the surface saturation variations. Since the conductivity is to ¢rst approximation proportional to the saturation at the surface, this might explain the observed phase relations observed in an experiment in a cave near Abaratsubo (Japan).

International audience; A time-dependent stochastic process with three states (solid, broken and moving) is considered in a hierarchical system made of embedded cells of increasing levels. An earthquake of a given scale k is associated with the moving state of a cell of level k and results from the coherent self-organization of fractures of lower scales. A direct cascade of stress redistribution generates small-scale stress heterogeneities in the neighbourhood of the active fracture. An interesting feature of the model is that the size of the domain where stress redistribution takes place grows proportional to the length of the fracture. In the framework of the general model, inspired by the progress in the use of the renormalization techniques in approaching critical point phenomena, we independently study a `fracturing' submodel and a `friction' submodel. These submodels are two-state models that act on different timescales. In the `friction' submodel, which comprises broken and moving states, the transitions between these two states are associated with stick-slip behaviour in a completely fractured fault zone. In the `fracturing' submodel, which comprises solid and broken states, we model the brittle behaviour of rock material. In both models we obtain a spatio-temporal clustering of earthquakes, realistic aftershock sequences whose frequency decreases respect the modified Omori law, and a frequency-magnitude relationship that respects the Gutenberg-Richter law. We show that the model behaviour is controlled by the stress heterogeneity in the fault zone, we find evidence for a relationship between the periodicity of the largest earthquakes and the b-value, and we indicate how the different physical ingredients underlying each submodel can be gathered together in a more general model.

Hypoganglionosis of the myenteric plexus of the colon is not clearly defined and seldom investigated. Colon segments from 15 children with an extended oligoeuronal hypoganglionosis up to the proximal resection end were morphometrically studied and compared to normally innervated colon segments. The study was performed with resected specimens from 7 children with isolated hypoganglionoses, 8 children with a Hirschsprung-associated hypoganglionosis, and 12 colon segments with normal innervation. The resected colon specimens were caudo-cranial coiled. The native tissue was frozen at -80 degrees C on a cryostat carrier and cut at -20 degrees C in 15 microns-thick sections (equivalent to 4-5-micron-thick paraffin sections). The air-dried sections underwent an enzyme-histochemical procedure for an acetylcholinesterase reaction to stain the parasympathetically innervated myenteric plexus. For histological identification and morphometric measurements, ganglia and nerve cells were selectively stained using a lactic dehydrogenase reaction. The morphometric measurements were performed with an optic-electronic image analysis system that determined ganglion size, ganglion distances, nerve cell number per ganglion, and ganglion number per mm colon. The results showed that hypoganglionosis of the myenteric plexus is characterised by a 42% decrease in plexus area and a 55% decrease of the nerve cell number per mm length of colon. The number and area of myenteric ganglia showed a decrease of 59% and a doubling of the ganglion distances. The histopathological diagnosis of a hypoganglionosis of the colon was not necessarily an indication of a chronic constipation, but rather an indication of a disposition for constipation. A chronic constipation is often caused by a long hypoganglionic segment proximal to a resected short Hirschsprung segment.

A prospective study of 141 consecutive patients with intestinal neuronal malformations is presented. The single malformation of the autonomic nervous system that always required surgical intervention was aganglionosis. Giant ganglia, reduced parasympathetic tone, immature ganglia, and hypogenetic or heterotopic nerve cells were seen in all forms of malformations. However, the incidence in specific malformations was variable. Multiple giant ganglia were identified in all patients with intestinal neuronal dysplasia (IND) type B, but also in various other malformations. Heterotopic nerve cells in the myenteric plexus were seen in the proximal segment of 15 of 74 patients (20.3%) with aganglionosis and 5 of 9 patients (55.6%) with hypoganglionosis. A significant impact on symptoms was found for IND type B: 34 (45.9%) of 74 children with aganglionosis had associated IND type B, and these children more frequently developed ileus (P0.001) and more often needed a second resection (P0.05) compared to those with isolated aganglionosis. This indicates an additive effect of both malformations, and therefore, in these patients an extended resection should be carried out. Twelve of 67 patients (17.9%) without aganglionosis needed resection for untreatable constipation. This included 7 of 9 children with hypoganglionosis, both patients with heterotopia of the myenteric plexus, 1 of 20 with isolated IND type B, and 2 of 12 with reduced parasympathetic tone. None of the patients with immaturity, heterotopia of the submucous plexus, or mild dysganglionosis required surgery. Six children (8.9%) without aganglionosis underwent sphincteromyotomy and 2 with IND type B had a temporary colostomy. At follow-up (mean 2.4 +/- 1.4 years), the outcome in patients with resected aganglionosis was better than in patients who had resections for other malformations; 49 (69%) of 71 patients with aganglionosis were asymptomatic compared to 4 (33.3%) of 12 with other malformations (P0.05). It is concluded that some intestinal malformations have a relevant clinical impact. However, the severity of symptoms in the individual patient may not be explained by specific histochemical findings from a limited number of mucosal biopsies. The pathognomonic histochemical criteria of isolated IND type B - immaturity, reduced parasympathetic tone, heterotopia of the submucous plexus, and mild dysganglionosis - rarely require surgical therapy and should be treated conservatively.

Beim erstmals 1972 beschriebenen Townes-Brocks-Syndrom handelt es sich um ein Dysmorphiesyndrom mit folgenden Merkmalen unterschiedlicher Penetranz: Analstenose, Analatresie, Innenohrschwerhorigkeit, triphalangeale Daumen oder Daumendoppelungen, Ohrmuscheldysplasie oder Ohranhangsel. Es wird eine Familie mit 3 erkrankten Mitgliedern (Mutter, Tochter, Sohn) vorgestellt, bei denen sich die Hauptmerkmale des Syndroms in unterschiedlicher Auspragung finden. Der in der Literatur zitierte Erbgang lies sich im Familienstammbaum in 2 Generationen nachvollziehen.

Neben der klassischen Aganglionose (Morbus Hirschsprung) und der Hypoganglinose sind in den letzten Jahren insbesondere durch die Arbeiten von Meier-Ruge zahlreiche weitere neuronale intestinale Fehlbildungen beschrieben worden. Hierzu gehoren insbesondere die neuronale intestinale Dysplasie, Reifestorungen der intestinalen neurogenen Strukturen, Storungen des bindegewebigen Grundgerustes der Darmwand mit Verodung der Plexusloge und andere. Alle Veranderungen konnen isoliert oder in Kombination auftreten. Sie sind in sehr unterschiedlichem Ausmas mit Motilitatsstorungen korreliert, die zu Ileus, einer chronischen Obstipation oder Uberlaufinkontinenz fuhren konnen. Nur die Zusammenschau der klinischen Symptomatik mit den Ergebnissen der radiologischen Defakographie, der Manometrie, Transitzeitstudien und den histologisch/histochemischen Befunden ermoglicht es, den wirkungsvollsten konservativen oder operativen therapeutischen Weg einzuschlagen.

The innervation patterns of the rectal pouch and fistula of 52 children with anorectal malformations were investigated. Posterior sagittal anorectoplasty was used for intermediate and high anomalies; for the latter it was combined with an abdominal approach. Perineoproctoplasty was performed for low anomalies. The specimens were investigated by acetylcholinesterase staining, lactate dehydrogenase, and succinyldehydrogenase reaction. They consisted of fistula material only in 23 patients and of parts of the rectal pouch in 29. Fourty-four patients (84.6%) had follow-up, and information of bowel movements and continence was obtained after a mean of 3.3 years. Abnormal innervation patterns were found in 96% of the specimens. All fistulas were found to be aganglionic, including the adjacent part of the rectum involving the internal sphincter equivalent. Classical aganglionosis was found in 31% of the rectal pouch specimens, hypoganglionosis in 38%, neuronal intestinal dysplasia (NID) type B in 14%, and dysganglionosis in 10%. All patients with severe constipation or soiling at the time of follow-up had some histopathological correlation. Of the 25 patients for whom the specimens had consisted of rectal pouch material, nine (31%) had severe constipation. All four patients with a low-type malformation who had follow-up and pathological innervation patterns in the rectal pouch suffered from severe constipation; this was true of only five of the 19 children with intermediate or high malformations (P.05). However, numerous pathological innervation patterns had been identified in patients who had normal bowel function at the time of follow-up. It is concluded that partial denervation of the rectum may not be the only cause in the pathogenesis of constipation after posterior sagittal anorectoplasty and perineoproctoplasty. The high frequency of neuronal intestinal malformations in the rectal pouch may be related to the higher frequency of bowel disturbances in patients with low malformations, in whom the resection was less radical. However, the clinical course is not necessarily related to specific histopathological findings. In the authors' opinion, the recommendation to use the distal rectal pouch and parts of the fistula in the reconstruction of anorectal malformations should be reconsidered.

Out of a series of 146 patients with oesophageal atresia 9 (6.2%) underwent colon interposition from 1963 to 1971. All eight surviving patients were seen at follow-up after a mean of 22 years. Three patients were free of specific symptoms according to the criteria of DeMeester, two had moderate and three severe distress. The mean time for consuming a standardized test meal was 15 minutes, compared to 8 minutes in healthy controls. Patients required 1-9 minutes to transport liquid barium through the transplant, compared to < 10 seconds in control subjects. Histological evaluation revealed a normal architecture of the colonic and ileal epithelium in three patients who underwent endoscopy. In none of these patients were contractions in the colon graft related to the act of swallowing recorded on manometry. Unimpaired quality of life was indicated by the Spitzer index which scored a mean of nine out of ten points. However, on a 100point visual analogue scale patients scored their global quality of life 66 and the mean Gastrointestinal Quality of Life Index was 92.2, compared to 107.6 in healthy control subjects (p < 0.05). This impairment was exclusively due to specific symptoms which scored 49.3 in patients and 59 in healthy individuals (p < 0.05). Physical and social functions, emotions, and inconvenience of a medical treatment were similar to control subjects. We conclude that colon interposition for long-gap oesophageal atresia achieves acceptable long-term functional results. However, specific symptoms lead to a considerable impairment in quality of life.

In order to examine variations in aftershock decay rate, we propose a Bayesian framework to estimate the {K, c, p}-values of the modified Omori law (MOL), lambda(t) = K(c + t)(-p). The Bayesian setting allows not only to produce a point estimator of these three parameters but also to assess their uncertainties and posterior dependencies with respect to the observed aftershock sequences. Using a new parametrization of the MOL, we identify the trade-off between the c and p-value estimates and discuss its dependence on the number of aftershocks. Then, we analyze the influence of the catalog completeness interval [t(start), t(stop)] on the various estimates. To test this Bayesian approach on natural aftershock sequences, we use two independent and non-overlapping aftershock catalogs of the same earthquakes in Japan. Taking into account the posterior uncertainties, we show that both the handpicked (short times) and the instrumental (long times) catalogs predict the same ranges of parameter values. We therefore conclude that the same MOL may be valid over short and long times.

Different GRACE data analysis centers provide temporal variations of the Earth's gravity field as monthly, 10-daily or weekly solutions. These temporal mean fields cannot model the variations occurring during the respective time span. The aim of our approach is to extract as much temporal information as possible out of the given GRACE data. Therefore the temporal resolution shall be increased with the goal to derive daily snapshots. Yet, such an increase in temporal resolution is accompanied by a loss of redundancy and therefore in a reduced accuracy if the daily solutions are calculated individually. The approach presented here therefore introduces spatial and temporal correlations of the expected gravity field signal derived from geophysical models in addition to the daily observations, thus effectively constraining the spatial and temporal evolution of the GRACE solution. The GRACE data processing is then performed within the framework of a Kalman filter and smoother estimation procedure. The approach is at first investigated in a closed-loop simulation scenario and then applied to the original GRACE observations (level-1B data) to calculate daily solutions as part of the gravity field model ITG-Grace2010. Finally, the daily models are compared to vertical GPS station displacements and ocean bottom pressure observations. From these comparisons it can be concluded that particular in higher latitudes the daily solutions contain high-frequent temporal gravity field information and represent an improvement to existing geophysical models.

We will show how to analyse the local regularity of functions with the help of the wavelet transform. These results will be applied to the function of Riemann, where we show the existence of a dense set of points where this function is differentiable. On another dense set we show the existence of local singularities of cusp type. On a third set we show differentiability to the right (left). On the remaining set the functions will be shown to be not differentiable.

We propose a new geometrical interpretation of two-dimensional turbulence energy spectrum, supposing the presence of at least one cusplike axisymmetric coherent structure in the flow. Such a coherent structure is a scaling distribution of vorticity which presents, instead of a characteristic radius, a range of radii corresponding to all scales where the associated energy spectrum has a power law behaviour. We compute a relation between the spectral slope and the exponent of the singularity in the Euler limit (Reynolds number tending to infinity). We predict a sensitivity of the two-dimensional flow dynamics to the regularity of the initial vorticity field: if it is initially regular, i.e. smooth, the coherent structures formed will be regular presenting a flat core, and if it is initially nonregular they will have a cusplike shape with only the vortex core regularized by dissipation, which corresponds to quasi-singularities.

In the past 25 years, from 1963 to 1988, 90 children from the Department of Pediatric Surgery of the Pediatric Hospital in Cologne, Germany were treated for biliary atresia. Of these, 47 had purely extrahepatic bile duct lesions, 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic bile duct lesions. Forty-five of the children underwent a drainage operation, whereas the remaining 45 children underwent no surgery at all or simply a diagnostic laparotomy. Until 1966 hepato-jejunostomy with implantation of artificial bile ducts was conducted in 12 cases. Later, cholecystoduodenostomy was performed 4 times and hepatoporto-jejunostomy according to Kasai-Kimura 29 times. The latter was performed either without and enterostomy (n = 16) or with an enterostomy in the respective intestinal loop (n = 13). 27 patients survived (30%). If only the children with intrahepatic bile duct hypoplasia are considered, the survival rate was 12 out of 15 patients (80%). Eight children (27.5%) of the 29 with hepatoporto-jejunostomy are still alive today. This survival rate, compared with the survival rate of the total, is comparably large with 27.7%. Current data from the 23 surviving patients was retrospectively gathered in our hospital or was collected from outside the establishment. It was analyzed with regard to prognosis and long-term results. Only in 5 of the 29 cases of children with hepatoporto-jejunostomy could a lasting postoperative biliary flow be achieved. Only one of the children can be classified as completely healthy in regard to his liver.(ABSTRACT TRUNCATED AT 250 WORDS)

101 patients were subjected to partial lung resection from 1962-1969 at the Division of Paediatric Surgery in the Department of Paediatrics of the University of Cologne. Follow-up examinations were performed in 38 patients. 24 patients were entirely without complaints, 9 had mild subjective complaints, and only 5 patients stated they were suffering continually from dyspnoea. Lung function tests were conducted in all patients, revealing a significant drop in vital capacity as the number of resected segments increased. The intrathoracic gas volume expressed in percentage of the standard value increased significantly with the number of resected segments. The flow volume curves dropped with the number of resected segments, whereas the airway resistance increased. These studies show that lung resections are not always tolerated without restricted function even by children. Hence, lung resection should be performed with utmost discretion and only if absolutely necessary. This is all the more important since adjacent pulmonary tissue may also be involved in the pathological process due to the underlying disease.

The clinical pattern of signs and symptoms of respiratory complications due to flaccid trachea has been analysed in 83 children treated in our hospital between 1983 and 1988 for tracheo-oesophageal malformations. These signs and symptoms are classified according to endoscopic findings, and are thus arranged according to various degrees of severity. Of the surviving children who were followed up and who were suffering from oesophageal atresia Vogt III B, only 5 of 57 were without a pointer towards flaccid trachea, whereas in oesophageal atresia Vogt II there were two of three. The two children with an isolated tracheo-oesophageal fistula showed abnormal findings both clinically and via endoscopy. 16 of the 20 children with very severely pronounced flaccid trachea--defined by the occurrence of life-threating apnoeas and an endoscopically identifiable tracheal collapse of more than two-thirds of the lumen--were subjected to surgery via aortosternopexy. A marked and identifiable improvement was obtained in 15 cases. Complications caused by surgery consisted of temporary phrenicus lesions in two cases.

The histopathology of Hirschsprung’s disease is defined by a complete absence of intramural nerve cells and a concomitant hypertrophy of nerve fascicles in caudal regions of the gastrointestinal tract. These pathomorphological criteria are readily recognized by standard enzyme and immunohistochemical techniques in establishing the diagnosis of Hirschsprung’s disease. In contrast, electron microscopic studies are more time-consuming and require several procedures prior to the assessment of the specimens (e.g. fixation, postfixations, semi- and ultrathin sectioning, and specific stainings with heavy metal compounds). Therefore, in clinical routine, electron microscopy is not the tool of first choice to confirm the histopathology of Hirschsprung’s disease.

Background In some patients, an initial pull-through procedure for Hirschsprung's disease fails, and obstructive symptoms persist or recur. Then a repeated pull-through operation may be necessary. Methods Seventeen patients with Hirschsprung's disease aged 2 to 9 years (median, 4.6 years) have undergone a repeated pull-through procedure because of unresponsive symptoms after an initial operation. The initial procedure was Soave in 3 patients, Rehbein in 13 patients, and Duhamel in 1 patient. Surgical revision was indicated by incomplete resection of the transition zone in 16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients. All 17 patients have undergone Redo Duhamel pull-through procedure. Median follow-up after Redo operation was 9 years (range, 1-23 years). Results In 15 patients, the stooling pattern normalized immediately after Redo procedure. Two, including 1 with Down's syndrome, are prone to constipation with occasional use of laxatives. Soiling is seen in the patient with Down's syndrome, but only with episodes of diarrhea. In spite of large formation of scars surrounding the neoanorectum in most patients, Duhamel pull-through reconstruction was possible in all children of this series. Conclusions The predominant cause for persistent or recurrent unresponsive obstructive symptoms after initial pull-through procedure is incomplete resection of the transition zone. Less frequently, anastomotic strictures, rigidity of the anorectal cuff, and fistulas cause obstruction. Preoperative workup must focus on these complications. The courses after initial pull-through procedure show that laxatives, Malone procedure, dilatations of the anorectum, myectomy, V-Y-plasty, and injections of botulinum toxin cannot eliminate the mechanical or functional obstruction. Although a large formation of scars in the pelvis resulting from the initial operation, myectomies, dilatations, and other surgical modalities render a Redo revision more difficult, Redo Duhamel pull-through procedure is able to provide the definitive solution to the problem.

Extreme hydrological events are often triggered by exceptional co-variations of the relevant hydrometeorological processes and in particular by exceptional co-oscillations at various temporal scales. Wavelet and cross wavelet spectral analysis offers promising time-scale resolved analysis methods to detect and analyze such exceptional co-oscillations. This paper presents the state-of-the-art methods of wavelet spectral analysis, discusses related subtleties, potential pitfalls and recently developed solutions to overcome them and shows how wavelet spectral analysis, if combined to a rigorous significance test, can lead to reliable new insights into hydrometeorological processes for real-world applications. The presented methods are applied to detect potentially flood triggering situations in a high Alpine catchment for which a recent re-estimation of design floods encountered significant problems simulating the observed high flows. For this case study, wavelet spectral analysis of precipitation, temperature and discharge offers a powerful tool to help detecting potentially flood producing meteorological situations and to distinguish between different types of floods with respect to the prevailing critical hydrometeorological conditions. This opens very new perspectives for the analysis of model performances focusing on the occurrence and non-occurrence of different types of high flow events. Based on the obtained results, the paper summarizes important recommendations for future applications of wavelet spectral analysis in hydrology.

Background Intestinal neuronal dysplasia (IND B) is still a subject of controversy. The aim of this paper is to review the present state of knowledge on IND B. A summary is given of the technical and diagnostic criteria which have to be considered in order to arrive at a reliable diagnosis. In addition, the available therapeutic interventions are discussed. Methods Between 1992 and 2001, 3984 colonic mucosal biopsies from 1328 children were investigated. Nerve cell staining was performed on native tissue sections: 15 microm thick cryostat sections, which, after spreading and drying on a microscopic slide, have a final thickness of 4-5 microm, with dehydrogenase reactions (lactic dehydrogenase, nitroxide synthase, succinic dehydrogenase). The biopsies were taken 8-10 cm above the dentate line (proximal to the ampulla recti, because of the caudo-cranial increase of giant ganglia proximal to the 4 cm biopsy) with a sufficient amount of submucosa. The criteria for IND is 15-20 % submucosal giant ganglia with more than eight nerve cells in 30 sections of a single biopsy (i.e. four to seven giant ganglia). Results The diagnosis of IND B is quantitative. A diagnosis of IND B was made over the past 10 years in 51 Hirschsprung resections (about 5 per year; 6 % of all Hirschsprung cases), and in 92 children with chronic constipation (about 9 children per year; 2.3 % incidence). Up to their fourth year of life, most children with isolated IND can be treated conservatively. This is due to the delayed maturation of the enteric nervous system which is characteristic of IND B. Only children who showed an additional hypoplastic hypoganglionosis were treated surgically. Children with Hirschsprung's disease (HD) and IND B proximal to the aganglionosis often showed, in those cases with a disseminated IND, postoperative disturbances in intestinal motility. Conclusion The diagnosis of IND B requires that biopsies are taken proximal to the ampulla recti (about 8-10 cm above the dentate line) with a sufficient amount of submucosa. The biopsies must be cut rectangular to the surface of the mucosa. A diagnosis of IND B can be made only if, in the submucosa of 30 serial sections, 15-20 % of all ganglia are giant ganglia with more than eight nerve cells. Ganglioneuromatosis (MEN2B) must be clearly differentiated from IND. The clinical course of IND B depends on the extent of disturbed bowel innervation, the severity of motility failure, and the coexistence of MH. The conservative management of isolated IND is possible in most children. In individual cases, however, a transient enterostomy or a segmental resection is unavoidable.

Vorgestellt wird ein Kind mit den typischen Symptomen eines Cat-eye-Syndroms jedoch ohne das namengebende Leitsymptom der Katzenaugen. Eine Chromosomenanalyse (47,XX- + mar de novo) ergab den Nachweis eines Markerchromosoms. Durch In-situ-Hybridisierung konnte nachgewiesen werden, das es sich bei dem vorliegenden Markerchromosom um Material vom Chromosom 22 handelt. Das Vollbild des charakteristischen Cat-eye-Symptomen-Komplexes findet sich nur in der Minderzahl der in der Literatur erwahnten Falle, jedoch gibt es bislang nur 2 Einzelbeobachtungen ohne Augenveranderungen. Als Erklarung fur die grose Variabilitat des Phanotyps sind unterschiedliche Grosen des vorhandenen Markerchromosoms und asymmetrische Bruchpunkte auf dem langen Arm des Chromosoms 22 denkbar. Nach neueren Untersuchungen ist die Variabilitat des Phanotyps allerdings nicht mit der Grose des Markers korreliert.

We report a patient presenting with severe muscular impairment and chronic intestinal pseudo-obstruction (CIP) at the age of eight months. Due to the aggravated symptoms, assisted ventilation, an ileostomy and total parenteral nutrition were required. Later on, the patient developed a locked-in syndrome (Leigh's subacute necrotising encephalomyelopathy) and finally died due to recurrent pneumonia and chronic renal failure. The assessment of muscle biopsies revealed a moderate single-fibre type II atrophy, a variation of muscle fibre calibre with focal fatty degeneration and a decreased reactivity of cytochrome-c oxidase. Although ragged red fibres had not been found, mitochondrial enzyme activities were markedly decreased with the lowest residual activity detected for NADH:Q1 oxidoreductase and NADH:O2 oxidoreductase (complex I deficiency), thereby confirming the diagnosis of mitochondrial myopathy. A molecular genetic analysis could not identify known mutations of mitochondrial DNA. Gastrointestinal full-thickness biopsies revealed myenteric hypoganglionosis of the colon and stomach and hyperplasia of the submucosal plexus of the ileum. Some of the intestinal smooth muscle cells displayed bulbous protrusions filled with lateralised mitochondria. Mitochondrial myopathies are known to be associated with a variety of clinical syndromes including CIP. However, in contrast to previous reports in which CIP has been attributed to visceral intestinal myopathies, the present case is characterised by neuronal intestinal malformations. Therefore, a mitochondrial myopathy associated with CIP requires a subtle assessment of both the intestinal smooth muscle and the enteric nervous system to identify the underlying pathology.

But: Par opposition a d'autres pays, aucune etude collective de la technique de Rehbein n'a ete realisee dans les pays de langue allemande. Ainsi, notre intention a ete, de maniere analogue a Goto et Ikeda, une etude Japonaise de 1984, a Kleinhaus une etude sur le procede de Swenson en 1979, a Bourdelat une etude franco-canadienne sur la technique de Duhamel en 1997, et des etudes de Martucielo et Teitelbaum avec un suivi jusque dans les annees 2000, de realiser une etude sur la technique de Rehbein. Methodes: Les informations de 200 patients de 22 centres parlant allemand en Suisse, Autriche et Allemagne ont ete reunis. Les informations ont ete collectees par questionnaire, et les enfants etaient suivis dans les differentes equipes participantes au moins 3 ans apres l'intervention chirurgicale. L'intervention a ete realisee entre 1993 et 1997 sur une periode de 5 ans. Le questionnaire contenait 74 items contenant des informations sur l'anamnese, le diagnostic, le traitement post-operatoire et les reinterventions. Resultats: Concernant l'incidence des fuites anastomotiques et des stenoses anastomotiques spontanement resolutives, il n'y a aucune difference significative entre les resultats de notre serie et ceux des analyses collectives faites par Hofmann von Kap-herr, Holschneider et Sherman. Chez 6.6% des 191 patients une fuite anastomotique et 9.9% de stenose rectale, qui ont du etre dilatees, ont ete observees. Concernant les complications plus tardives, 22.8% des enfants souffraient de constipation, 4.2% d'encopresie, 10.6% d'enterocolite, et seulement 0.5% d'enuresie. La frequence de la constipation diminue annee apres annee. Une comparaison des differentes grandes series de la litterature montre clairement que l'incidence de la constipation est plus elevee apres l'intervention de Rehbein et que l'incontinence urinaire et l'encopresie sont plus elevees apres les techniques de Swenson, Soave et Duhamel. L'incidence de l'enterocolite est moindre apres l'intervention de Rehbein qu'apres les techniques de Swenson, Soave et Duhamel. Conclusion: Les differents resultats de la litterature sont dus a l'experience individuelle de chaque auteur avec des differences notables concernant les methodes et la periode de suivi. Par consequent, les differents resultats sont difficiles a comparer. Neanmoins, la resection anterieure de Rehbein peut etre presentee comme une technique adequate et importante pour traiter la maladie de Hirschsprung.

Posterior sagittal anorectoplasty provides an optimal access to reconstruct the muscle complex in anorectal malformations. It gives much better results than the abdominoperineal pullthrough procedures performed before 1984. However, severe chronic constipation occurs postoperatively in about 10 % of the patients, which can only be treated by washouts. Clinical investigations of 578 patients treated from 1962 to 1984 and from 1985 to 1997 are presented here and both groups are compared to each other. In addition, a new continence score with special regard to chronic constipation and overflow incontinence was used to follow up 133 patients of the second group. The score distinguishes between children above and below the age of 3 years. To study the underlying reasons of severe chronic constipation in children with anorectal malformations, macro- and microanatomical investigations on 4 normal newborns, 3 neonatal piglets with imperforate anus and 25 rectal biopsies from the caecum were performed. The following reasons have been found to be probably responsible for postoperative constipation and overflow incontinence: 1. Malformations of the smooth and striated muscle fibres or connective tissue of the caecum; 2. Malformations of the intramural nerve plexus such as aganglionosis, hypoganglionosis or IND; 3. Malformations and/or iatrogenic lesions of the extramural nerve supply which runs anterior to the rectum and in front of the fascia of Denonvilliers, which can hardly be identified in neonates with imperforate anus. Therefore iatrogenic bladder injuries may occur after PSARP after extended mobilisation of the caecum. The macro- and microanatomical situation in the piglet with imperforate anus is totally different from the human newborn.