Biliary atresia (BA) is a rare condition in newborns characterised by inflammation and obliteration of bile ducts, leading to cirrhosis. Early diagnosis and treatment through Kasai portoenterostomy are crucial for native liver survival. While diagnostic methods include clinical assessment, laboratory tests and imaging, no non-invasive test can definitively rule out BA. Therefore, laparotomy with cholangiography remains the gold standard for diagnosis. This study assesses the feasibility, safety and accuracy of endoscopic retrograde cholangiopancreatography (ERCP) for diagnosing BA in newborns. The single-center study was conducted at the University Hospital Tübingen. The study included infants with neonatal cholestasis and suspected BA scheduled for ERCP from 2011 to 2023. We identified 60 jaundiced infants with suspected BA scheduled for diagnostic ERCP with a median age of 50 days. Endoscopy was technically feasible in 58/60 children. In 23 of 58 cases, the bile ducts could not be visualized, suggesting BA. Normal bile duct anatomy was visualized in 35/58 patients, and BA was ruled out. The sensitivity was 100
Background: The classification of fractures is necessary to ensure a reliable means of communication for clinical interaction, research, and education. Distal humeral coronal plane shear fractures (CSF) are very rare in the growing-age population, despite that multiple classification schemes exist. Objective: The aim of this study was to assess the inter- and intra-rater reliability of the 4 most commonly used CSF classifications and their role in guiding treatment decisions in everyday clinical practice. Materials and methods: 51 patients with CSF were retrospectively analyzed in a multicenter study. Of these, X-rays in two planes of 20 randomly selected CSF were given to 8 independent raters for classification according to Dubberley, Bryan & Morgan, Murthy, and AO. Cohens Kappa statistic was used to assess inter-rater agreement and intra-rater consistency. Intra-class correlation coefficient (ICC) estimates and 95% confident intervals (CI) were calculated. Reliability values were classified as excellent (ICC ≥0.8), substantial (ICC 0.61-0.80), moderate (ICC 0.41-0.6), fair (ICC 0.2-0.4), slight (ICC 0.00-0.2), and poor (ICC <0.00). Results: All patients received x-rays in 2 planes initially. Cross-sectional imaging was added in 76.5% of cases (CT 58,8%, MRI 11,7%, both 5,9%). Inter-rater reliabilities were classified as fair for Dubberley (ICC 0,354; 95% CI 0,198; 0,573) and Bryan & Morgan (ICC 0,357; 95% CI 0,200; 0,576), slight for AO (ICC 0,226; 95% CI 0,100; 0,434), and poor for Murthy (ICC -0,012; 95% CI -0,063; 0,102). The Dubberley subtype showed slight agreement at lower ICC values (ICC 0,024; 95% CI -0,041; 0,161). Intra-rater agreement was moderate to substantial for most of the 8 raters, and did not differ between the four classification systems. The rate of surgical treatment was 49/51 patients. Conclusion: The most commonly used classification schemes for CSF failed to achieve a substantial agreement among the raters. This is probably because the fracture patterns of CSF in adolescence and high adulthood differ significantly. An adaptation of the classification for pediatric and adolescent patients is necessary, but only for scientific purposes. Classification is not necessary for the clinical management of patients, as virtually all patients require surgical treatment.
The management of congenital lung malformations (CLM) remains controversial. The aim of this study was to analyze the timing, approach, and outcomes of surgical treatment in children with CLM.A cohort study was conducted comparing children with CLM who underwent thoracoscopic resection with open resection via thoracotomy. All children were treated according to an institutionalized multidisciplinary coordinated treatment algorithm.Between 2002 and 2019, 68 children with CLM were treated. Thoracoscopic resection of CLM (n=44) resulted in a statistically significant longer operative time (mean 169 vs. 97 minutes, p=0.04) but shorter hospital stay (mean 7.2 vs. 16.7/12 days, p=0.01) compared to thoracotomy (n=15) or after conversion to thoracotomy (n=9). There were no major complications in either group. At a mean follow-up of 32.2 months (range 0.5-163), patients after thoracoscopic resection had statistically significantly fewer rip fusions than patients after open resection (n=0 vs. 2/2, p≤0.004) and less chest wall asymmetry than after conversion (n=0 vs. 2, p=0.004).Thoracotomy proved to be a fast and safe surgical approach in respiratory unstable CLM patients. In respiratory stable CLM patients, thoracoscopic resection was feasible in early infancy with good surgical and musculoskeletal outcomes.Thoracoscopic resection of CLM offers advantages over resection by thoracotomy in terms of surgical recovery and musculoskeletal function. Therefore, it should be offered to respiratory-stable patients in specialized centers.
In the past decade, photoemission orbital tomography (POT) has evolved into a powerful tool to investigate the electronic structure of organic molecules adsorbed on surfaces. Here we show that POT allows for the comprehensive experimental identification of all molecular orbitals in a substantial binding energy range of more than 10 eV. Making use of the angular distribution of photoelectrons as a function of binding-energy, we exemplify this by extracting an orbital-resolved projected density of states for 15 pi and 23 sigma orbitals from the experimental data of the prototypical organic molecule bisanthene (C28H14) on a Cu(110) surface. These experimental results for an essentially complete set of orbitals within the given binding-energy range serve as stringent benchmarks for electronic structure methods, which we illustrate by performing density functional calculations employing four frequently used exchange-correlation functionals. By computing the respective molecular-orbital-projected densities of states, a one-to-one comparison with experimental data for an unprecedented number of 38 orbital energies became possible. The quantitative analysis of our data reveals that the range-separated hybrid functional HSE performs best for the investigated organic/metal interface. At a more fundamental level, the remarkable agreement between the experimental and the Kohn-Sham orbital energies over a binding-energy range larger than 10 eV suggests that-perhaps unexpectedly-Kohn-Sham orbitals approximate Dyson orbitals, which would rigorously account for the electron extraction process in photoemission spectroscopy but are notoriously difficult to compute, in a much better way than previously thought.
The approach of photoemission orbital tomography, i.e., the orbital density reconstruction from photoemission of planar molecular layers by using a formalism equivalent to a Fourier transformation, is transferred to free atoms. Absolute radial orbital densities of neon 1s, 2s, and 2p orbitals are reconstructed with a central-field one-electron model, using well-known atomic photoionization data. The model parameters are optimized by a Markov chain Monte Carlo method with Bayesian inference from which uncertainties for the reconstructed orbital densities are derived. The presented model opens the path for photoemission orbital tomography as a powerful tool, as well as for a quantitative analysis.
Photoemission spectroscopy is used to quantitatively determine the partial cross section of the 1t2 and 2a1 subshells of methane. Photoelectron spectra are recorded using a hemispherical electron analyzer and monochromatized synchrotron radiation. The corresponding emission peaks are analyzed to calculate the partial cross sections with the associated uncertainties in a photon energy range from 16.5 to 70 eV. The presented results cover this broad energy range with corresponding uncertainties and belong to the framework of photoemission orbital tomography, aiming to bridge the experimental gap between simple single atoms and complex molecular monolayers.
Background Treatment of unstable forearm fractures in the metaphyseal-diaphyseal junction (MDJ) zone is still a matter of debate. Major drawbacks of all types of fixations include either invasiveness, technical impracticality, or lack of acceptance by patients. This study reports results after antegrade ESIN (a-ESIN) compared to transepiphyseal intramedullary K-wire (TIK) for unstable MDJ forearm fractures. Methods The MDJ of the forearm was defined as the square over the joints of both forearm bones subtracted with the square over the metaphysis of the radius alone. The data of 40 consecutive patients < 16 years of age who were treated either by a-ESIN (later treatment period) or TIK (early treatment period) for an unstable MDJ forearm fracture at a single high-volume pediatric trauma center were retrospectively analyzed. Results The average age was slightly lower in the first group (TIK = 7.42 years; a-ESIN = 10.5 years). An additional ulna fracture was found in 50% of cases and was treated with a classic antegrade ESIN in 10/20 (TIK) and 6/20 cases (a-ESIN). Additional plaster cast immobilization was performed in all cases with TIK and in three cases with a-ESIN. After TIK, no complication, malalignment, or functional limitation occurred. After a-ESIN, 19/20 patients had an event-free course with stable retention and healing without axial malalignment. In one case, a temporary sensor dysfunction occurred. The same patient suffered a refracture two months after the original trauma, which required a closed reduction. Metal removal was performed after 84 days (TIK) and 150 days (a-ESIN). The outcome in all patients was good. Conclusion Both a-ESIN and TIK are minimally invasive procedures that are technically easy to perform. Both methods are safe and lead to a complete restoration of the forearm's range of motion. The decisive advantage of a-ESIN is the possibility of postoperative immobilization-free rehabilitation.
The approach of photoemission orbital tomography (POT), i.e., the orbital density reconstruction from photoemission of planar molecular layers, is applied to measurements of the partial cross section from gas-phase methane $({\mathrm{CH}}_{4})$. Within a central-field and one-electron model, the method previously validated on neon is tested on ${\mathrm{CH}}_{4}$'s molecular orbitals $2{a}_{1}$ and $1{t}_{2}$ with the goal to reconstruct absolute radial orbital densities. The reconstruction method is based on a Markov chain Monte Carlo optimization method, employing Bayesian inference, which additionally offers a reliable uncertainty evaluation. Applying the method to ${\mathrm{CH}}_{4}$, we aim to bridge the gap between simple single atoms and molecular layers, and to find a practical reference standard for the determination of orbital densities within POT.
Resection of lung metastases in children with solid tumors is regularly hampered by limited intraoperative detectability and relevant operative trauma of the open surgical access. The aim of this study was to analyze thoracoscopic resection of lung metastases in children following CT-guided labeling with coil wires. We retrospectively analyzed data of children and adolescents undergoing this approach at our institution between 2010 and 2022 with regard to technical aspects as well as surgical and oncological data. Within this period, we performed this procedure on 12 patients wherein we resected 18 lesions (1-5 per patient). The median age of patients was 178 months (51-265). The median duration of coil wire placement was 41 min (30-173) and the median surgery time was 53 min (11-157). No conversions were necessary and no intraoperative complications occurred. Complete microscopic resection (R0) was achieved in all labeled lesions and malignant tumor components were found in 5/12 patients. Our study shows that with a careful patient selection, thoracoscopic resection of lung metastases after coil wire labeling is a safe and reproducible procedure in children. Using this approach, lesions that are expected to have a reduced intraoperative detectability during open surgery become resectable. Patients benefit from the minimally invasive surgical access and reduced operative trauma.
Since May 2021, an extension of §1631 has been implemented in Germany: gender reassignment surgery in patients with differences of sex development (DSD) before the capacity for self-determined consent needs an interdisciplinary expert opinion and consent of the family court judge.The only exceptional cases are those in which the patient's health would be seriously endangered without such an operation.Congenital adrenal hyperplasia (CAH) is classified to the group of DSD, representing one of the DSD-groups with the highest incidence.Contrary to the current surgical procedure during the first two years of life, CAH girls should therefore grow up with virilized external genitalia until puberty and only then be operated on. Materials and methods:We report a case of an infant with CAH.The family originates from the Near East and immigrated to Germany only one year before the birth.The case was discussed in the DSD-commission with the aim of preparing an expert opinion for the family court. Results:At birth the child was initially assigned and named as male.After diagnosis of CAH with external genitalia Prader score V the family insisted on gender reassignment surgery.No urinary tract infections occurred, micturition was normal, endocrinological treatment was well tolerated without relevant reduction of the enlarged clitoris.Therefore, there were no medical reasons for early surgery, and initially, the commission failed to find agreement on recommendation for surgery.Finally, after intensive interdisciplinary follow-up of the parents and the child by psychologists, ethicists, social paediatricians, endocrinologists, and paediatric surgeons, a consensus decision was reached pro surgery.This was mainly based on the fact, that the psychological assessment showed that the family would reject the child due to virilization.The family court judge's approval was thereafter given quickly.Surgery was performed at the age of 23 months in form of a clitoral reduction and feminizing
Einleitung Die Gallengangsatresie ist eine seltene inflammatorische Cholangiopathie des Neugeborenen, die zu einer frühen Obliteration der Gallenwege mit sekundärer biliärer Leberfibrose führt. Die frühe Diagnose und adäquate Therapie (Portoenterostomie nach Kasai) sind für das Überleben mit der eigenen Leber essentiell. Die zeitgerechte Differentialdiagnostik ist bei den sehr jungen Säuglingen komplex: Anamnese, klinische Untersuchung, Labordiagnostik und Sonographie stellen die Grundlage in der Diagnostik, allerdings kann keine nichtinvasive Untersuchung eine Gallengangsatresie ausschließen, so dass weiterhin die Laparotomie mit offener Cholangiographie der Goldstandard in der Diagnostik der Gallengangsatresie ist. Hier stellt die endoskopische retrograde Cholangiopankreatikografie (ERCP) mit ihrer hohen fluoroskopischen Ortsauflösung eine wichtige interventionelle Alternative dar.
Angle-resolved photoemission spectroscopy (ARPES) is a method that measures orbital and band structure contrast through the momentum distribution of photoelectrons. Its simplest interpretation is obtained in the plane-wave approximation, according to which photoelectrons propagate freely to the detector. The photoelectron momentum distribution is then essentially given by the Fourier transform of the real-space orbital. While the plane-wave approximation is remarkably successful in describing the momentum distributions of aromatic compounds, it generally fails to capture kinetic-energy-dependent final-state interference and dichroism effects. Focusing our present study on quasi-freestanding monolayer graphene as the archetypical two-dimensional (2D) material, we observe an exemplary E_{kin}-dependent modulation of, and a redistribution of spectral weight within, its characteristic horseshoe signature around the K[over ¯] and K[over ¯]^{′} points: both effects indeed cannot be rationalized by the plane-wave final state. Our data are, however, in remarkable agreement with ab initio time-dependent density functional simulations of a freestanding graphene layer and can be explained by a simple extension of the plane-wave final state, permitting the two dipole-allowed partial waves emitted from the C 2p_{z} orbitals to scatter in the potential of their immediate surroundings. Exploiting the absolute photon flux calibration of the Metrology Light Source, this scattered-wave approximation allows us to extract E_{kin}-dependent amplitudes and phases of both partial waves directly from photoemission data. The scattered-wave approximation thus represents a powerful yet intuitive refinement of the plane-wave final state in photoemission of 2D materials and beyond.
Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.
Tracing the modifications of molecules in surface chemical reactions benefits from the possibility to image their orbitals. While delocalized frontier orbitals with π character are imaged routinely with photoemission orbital tomography, they are not always sensitive to local chemical modifications, particularly the making and breaking of bonds at the molecular periphery. For such bonds, σ orbitals would be far more revealing. Here, we show that these orbitals can indeed be imaged in a remarkably broad energy range and that the plane wave approximation, an important ingredient of photoemission orbital tomography, is also well fulfilled for these orbitals. This makes photoemission orbital tomography a unique tool for the detailed analysis of surface chemical reactions. We demonstrate this by identifying the reaction product of a dehalogenation and cyclodehydrogenation reaction.
In the past decade, photoemission orbital tomography (POT) has evolved into a powerful tool to investigate the electronic structure of organic molecules adsorbed on (metallic) surfaces. By measuring the angular distribution of photoelectrons as a function of binding energy and making use of the momentum-space signature of molecular orbitals, POT leads to an orbital-resolved picture of the electronic density of states at the organic/metal interface. In this combined experimental and theoretical work, we apply POT to the prototypical organic π-conjugated molecule bisanthene (C_28H_14) which forms a highly oriented monolayer on a Cu(110) surface. Experimentally, we identify an unprecedented number of 13 π and 12 σ orbitals of bisanthene and measure their respective binding energies and spectral lineshapes at the bisanthene/Cu(110) interface. Theoretically, we perform density functional calculations for this interface employing four widely used exchange-correlation functionals from the families of the generalized gradient approximations as well as global and range-separated hybrid functionals. By analyzing the electronic structure in terms of orbital-projected density of states, we arrive at a detailed orbital-by-orbital assessment of theory vs. experiment. This allows us to benchmark the performance of the investigated functionals with regards to their capability of accounting for the orbital energy alignment at organic/metal interfaces.
Nonsurgical management has been identified as the treatment of choice for femoral shaft fractures in children below four years of age. For various reasons, the surgical approach has become increasingly popular in recent years. The aim of this study is to report results after vertical skin traction and analyze the benefits of this technique as well as to point out advantages compared with surgery in this age group. The authors performed a retrospective data analysis, including all patients with femoral shaft fractures below the age of four who were treated with vertical skin traction at our institution between January 2006 and December 2016. Skin traction for a femoral shaft fracture was performed for 36 patients (mean age 1.6 years; 1 day–3.5 years). The mean duration of traction was 18.5 days (14–30). Complications included soft tissue affections (n = 5), which all healed spontaneously. Consolidation was observed in all fractures. Initial axial deviations and shortening did not change during traction until consolidation (p > 0.05), and no relevant torsion deformity occurred (p = 0.01). Patients gained full weight-bearing within 12.3 days (7–40) following end of traction. At the final follow-up, after a mean of 29.3 months (12–192), leg-length discrepancy (mean 7.1 mm; 5–20) was found on radiograms in nine cases, and axial deviations (mean 7.7°; 5–25) were documented in seven cases. None of the patients had limitations in daily activities or sports. Skin traction is a technically easy, safe, and non-invasive treatment modality for femoral shaft fractures in children below the age of four years. Strong results are obtained benefited from a high potential of growth-related correction, and in principle no anesthesia is needed. A prolonged hospitalization and socio-economic factors maintain the ongoing debate in comparison with the surgical approach. Level III, retrospective.
A gas detector system was implemented to monitor the absolute photon flux at the insertion device beamline of the Metrology Light Source. This system should overcome the drawbacks in the use of traceably calibrated semiconductor photodiodes, which are prone to radiation-induced degradation and not qualified for permanent real-time monitoring during the measurements at the beamline endstation. Gas monitor detectors are based on atomic photoionization. They do not suffer from radiation-induced degradation, and their transparency makes them suitable for real-time monitoring of the photon flux. We calibrated and commissioned such a monitor at the insertion device beamline in the vacuum- and extreme ultraviolet spectral range from 14 eV to 111 eV photon energy. The resulting photon flux measurement was compared to measurements made with a photodiode and shows deviations below 5 %, which agrees well with the calculated relative uncertainty of 4.7 %.
Polycyclic aromatic compounds with fused benzene rings offer an extraordinary versatility as next generation organic semiconducting materials for nanoelectronics and optoelectronics due to their tunable characteristics, including charge-carrier mobility and optical absorption. Nonplanarity can be an additional parameter to customize their electronic and optical properties without changing the aromatic core. In this work, we report a combined experimental and theoretical study in which we directly observe large, geometry-induced modifications in the frontier orbitals of a prototypical dye molecule when adsorbed on an atomically thin dielectric interlayer on a metallic substrate. Experimentally, we employ angle-resolved photoemission experiments, interpreted in the framework of the photoemission orbital tomography technique. We demonstrate its sensitivity to detect geometrical bends in adsorbed molecules and highlight the role of the photon energy used in experiment for detecting such geometrical distortions. Theoretically, we conduct density functional calculations to determine the geometric and electronic structure of the adsorbed molecule and simulate the photoemission angular distribution patterns. While we found an overall good agreement between experimental and theoretical data, our results also unveil limitations in current van der Waals corrected density functional approaches for such organic/dielectric interfaces. Hence, photoemission orbital tomography provides a vital experimental benchmark for such systems. By comparison with the state of the same molecule on a metallic substrate, we also offer an explanation why the adsorption on the dielectric induces such large bends in the molecule.
Background: Gastric transposition (GT) is a possible option for esophageal replacement in long-gap esophageal atresia (LGEA). The present study aims to report and compare indications and outcome of laparoscopic-assisted GT (LAGT) versus open (OGT) GT for LGEA repair. Methods: Retrospective single-center analysis of all LGEA patients undergoing GT between 2002 and 2021. Results: Thirty-one children with LGEA underwent GT. Of these, 19 underwent LAGT (mean weight at surgery 5.6 kg; mean age 167 days) and 12 underwent OGT (6.1 kg; 233 days). Indications for OGT were previous surgery (n = 7), associated severe cardiac malformations (n = 4), and a simultaneous resection of a choledochal cyst (n = 1). The conversion rate was 1. The two procedures (LAGT/OGT) differed in anesthetic time (308/350 min), duration of ventilation (5.1/5.3 days), hospital stay (34/32 days), and complications (22/15). None of the differences reached statistical significance. Outcome was also comparable: completely oral nutrition uptake in 66%/73%, slow weight gain in the low centiles in both groups, no patient developed dumping syndrome, symptomatic reflux was seen in 1 patient after OGT. Conclusion: In our cohort, LAGT for repair of LGEA provided similar outcomes as open surgery. The minimally invasive approach preserves thoracal structures, prevents additional thoracotomy or laparotomy, and is faster. To realize LAGT, a postpartal treatment concept including gastrostomy placement via a microincision to minimize adhesions is essential. The open surgical approach should be considered in cases of previous extensive surgical attempts of EA correction causing severe adhesions as well as associated anomalies or genetic syndromes causing hemodynamic instability.
Hexacene, composed of six linearly fused benzene rings, is an organic semiconductor material with superior electronic properties. The fundamental understanding of the electronic and chemical properties is prerequisite to any possible application in devices. We investigate the orientation and interface properties of highly ordered hexacene monolayers on Ag(110) and Cu(110) with X-ray photoemission spectroscopy (XPS), photoemission orbital tomography (POT), X-ray absorption spectroscopy (XAS), low-energy electron diffraction (LEED), scanning tunneling microscopy (STM), and density functional theory (DFT). We find pronounced differences in the structural arrangement of the molecules and the electronic properties at the metal/organic interfaces for the two substrates. While on Cu(110) the molecules adsorb with their long molecular axis parallel to the high symmetry substrate direction, on Ag(110), hexacene adsorbs in an azimuthally slightly rotated geometry with respect to the metal rows of the substrate. In both cases, molecular planes are oriented parallel to the substrate. A pronounced charge transfer from both substrates to different molecular states affects the effective charge of different C atoms of the molecule. Through analysis of experimental and theoretical data, we found out that on Ag(110) the LUMO of the molecule is occupied through charge transfer from the metal, whereas on Cu(110) even the LUMO+1 receives a charge. Interface dipoles are determined to a large extent by the push-back effect, which are also found to differ significantly between 6A/Ag(110) and 6A/Cu(110).